External Hydrocephalus Research Articles

Effectiveness and safety of Tachosil® as a ventricular sealant: an observational cohort study.

Surgery close to or in contact with the ventricular system is challenging due to the complications. We sought to evaluate the effectiveness and safety of TachoSil® as a ventricular sealant in preventing complications after cranial surgery with an open ventricular system (OVS). This is a single-center and prospective cohort study We included patients who underwent elective surgery for supratentorial craniotomy and periventricular pathology between December 2020 and November 2023. We registered surgical complications arising from CSF dynamics (such as percutaneous cerebrospinal fluid (CSF) leakage, hydrocephalus, pseudomeningocele), infections, and other complications (postsurgical hematoma) adverse drug reactions (ADRs), reintervention or hospital readmission up to 90days after surgery. Forty interventions were performed on 39 patients, whose median age was 56years. Eleven patients (28.2%) had antecedents of previous surgery in the same location, 5 (12.8%) had previously received radiotherapy and chemotherapy, and 11 (28.2%) were smokers. Twenty-four patients (60%) underwent surgery for high-grade glioma, 8 (20%) for low-grade gliomas, 6 (15%) for metastasis and 2 (5%) for meningioma. Throughout the study and up to 90days after surgery, none of the patients presented an ADR. Only 2 patients (5%) presented with a surgery complications derived from ventricular opening (one patient with a percutaneous CSF leakage and one patients with external hydrocephalus). Both patients resolved with a ventriculoperitoneal shunt. TachoSil® is a dural sealant that can be used safely and effectively intraparenchymally in patients whose surgery involves a ventricular opening. Only 5% of treated patients presented complications arising from CSF hydrodynamics. No patients had pseudomeningocele, infections or complications related to the use of this sealant. To confirm these positive results, randomized and comparative clinical trials assessing the efficacy of TachoSil® in patients after cranial surgery with an OVS are essential. This study was registered in the Clinical Trials.gov (NCT05717335). Date May 1st, 2022.

The postulated role of brain pulsations and arachnoid membranes in cerebrospinal fluid circulation, ventriculomegaly and related CSF disorders

Hydrocephalus can be obstructive or communicating. In cases of communicating hydrocephalus, patients will typically have enlarged brain ventricles. It has long been thought that the reason for this is obstruction to the outflow of cerebrospinal fluid from the subarachnoid space, which also results in raised intracranial pressure (ICP). However, there are several clinical and anatomic observations that are not well explained by this idea. These are for example, the conditions of Normal pressure hydrocephalus (NPH) which has normal ICP but ventriculomegaly, and idiopathic intracranial hypertension (IIH) which has raised ICP but normal sized ventricles. This hypothesis states that the mechanism for raised ICP and large ventricles seen in communicating hydrocephalus (ventriculomegaly) are different and seeks to explain the cause of ventriculomegaly using illustrative examples. It also suggests explanations for why ventriculomegaly occurs in NPH, infectious or carcinomatous meningitis, but is absent in IIH, or dural venous sinus thrombosis. Based on operative neurosurgical observations it states that the arachnoid membranes in the basal cisterns serve as part of a directional CSF flow mechanism consisting of fluid diodes, or “Tesla valves” of arachnoid membrane, containing CSF that is propelled by brain pulsations from the point of exit from the 4th ventricle to its points of absorption. This hypothesis suggests explanations for the physiologic appearance of a near uniform subarachnoid space, as well as the occurrence of the syndrome of the trephined and external hydrocephalus seen following a decompressive craniectomy. Also suggested are avenues of further research testing with MRI phase contrast CSF flow studies, classification of anatomic and peri-operative observations and rationale for creation of novel experiments to test the hypothesis, and aid diagnosis and treatment of a number of cranial CSF disorders.

Jugular foramen stenosis in external hydrocephalus in infants.

To measure the size of jugular foramina in infants affected by external hydrocephalus (EH) and in a control group, to support the hypothesis that a jugular foramen (JF) stenosis may determine dural venous sinus alterations and increased venous outflow resistance as main pathophysiological factor. Minimum, maximum, and mean values of JF areas were measured in a series of phase-contrast magnetic resonance venous angiography (angio MRV PCA3D) performed on 81 infants affected by EH. Results were compared with a group of 54 controls. Smaller JF area was significantly smaller in patients versus controls (43.1 ± 14.6 vs. 52.7 ± 17.8; p < 0.001) resulting in a significantly smaller mean JF areas in patients vs. controls (51.6 ± 15.8 vs. 57.0 ± 18.3; p = 0.043). In patients, smaller JF areas were significantly associated with higher venous obstruction grading score (VOGS) both on the right (p = 0.018) and on the left side (p = 0.005). Positional plagiocephaly (cranial vault asymmetry index > 3.5%) was more frequent among EH patients than controls (38/17) but the difference was not significant (p = 0.07). In the 38 plagiocephalic patients, JF area was smaller on the flattened side than the contralateral in a significant number of cases both in right (21/7) and left (9/1) plagiocephaly (p < 0.0005) as well as the mean area (48.2 + 16.4 mm2 vs. 57.5 + 20.7 mm2, p = 0.002) and VOGS was significantly higher on the plagiocephalic side than on the contralateral side (1.6 ± 1.1 vs. 1.1 ± 0.9, p = 0.019). In this series of infants affected by EH, the mean size of the ostium of both JF resulted significantly smaller than controls. JF stenosis was significantly associated with higher degrees of venous obstruction on both sides, suggesting a direct extrinsic effect of JF size on dural sinus lumen and possible consequent effect on venous outflow resistance. Positional plagiocephaly, when present, was associated with a decreased JF area and increased VOGS on the flattened side.

Congenital/Primitive Hydrocephalus: Classification, Clinical Aspects, and Rehabilitation Approach

AbstractHydrocephalus is a heterogeneous disorder of cerebrospinal fluid (CSF) flow that leads to abnormal enlargement of the brain ventricles. The prevalence of infant hydrocephalus is approximately one case per 1,000 births. Hydrocephalus occurs due to an imbalance between the production and the absorption of CSF. The causes of hydrocephalus secondary to CSF overproduction are papilloma of the choroid plexus and rarely diffuse hyperplasia of the villi. All the other hydrocephalus forms are secondary to obstruction to normal CSF reabsorption and are also known as obstructive hydrocephalus. According to the location of obstruction, obstructive hydrocephalus can be defined as communicating, when caused by extraventricular obstruction of the CSF flow or decreased resorption of CSF distal to the fourth ventricle in the cisterns of the base or in the subarachnoid spaces, or as not communicating, in case of intraventricular obstruction to fluid flow. There is a third category, common in preterm infants, called external hydrocephalus which is secondary to delayed development of arachnoid function. Hydrocephalus leads to an increase in intraventricular pressure because of the lack of the mechanism regulating the homeostasis of the CSF flow. Increased intraventricular pressure is responsible for the clinical symptoms in affected child. Clinical presentation varies with age. In the neonatal period, prolonged or frequent apneic or bradycardic events, increasing head circumference, presence of sunsetting eyes or upward gaze palsy, evidence of full or tense anterior/posterior fontanelle, and splayed cranial sutures are signs of increased intracranial pressure. In infants, the most common signs are progressive macrocephaly, irritability, nausea/vomiting, headache, gait changes, and regression of developmental milestones. The extent of brain damage depends on the cause that led to hydrocephalus, the patient's age, and the rapidity of onset. The surgical treatment modalities consist of endoscopic ventriculostomy of the third ventricle and ventriculoperitoneal or ventriculoatrial CSF shunt.

Erratum: Transcranial optical monitoring for detecting intracranial pressure alterations in children with benign external hydrocephalus: a proof-of-concept study

Erratum: Transcranial optical monitoring for detecting intracranial pressure alterations in children with benign external hydrocephalus: a proof-of-concept study

A novel de novo variant in CASK causes a severe neurodevelopmental disorder that masks the phenotype of a novel de novo variant in EEF2.

We report a 9-year-old Spanish boy with severe psychomotor developmental delay, short stature, microcephaly and abnormalities of the brain morphology, including cerebellar atrophy. Whole-exome sequencing (WES) uncovered two novel de novo variants, a hemizygous variant in CASK (Calcium/Calmodulin Dependent Serine Protein Kinase) and a heterozygous variant in EEF2 (Eukaryotic Translation Elongation Factor 2). CASK gene encodes the peripheral plasma membrane protein CASK that is a scaffold protein located at the synapses in the brain. The c.2506-6 A > G CASK variant induced two alternative splicing events that account for the 80% of the total transcripts, which are likely to be degraded by NMD. Pathogenic variants in CASK have been associated with severe neurological disorders such as mental retardation with or without nystagmus also called FG syndrome 4 (FGS4), and intellectual developmental disorder with microcephaly and pontine and cerebellar hypoplasia (MICPCH). Heterozygous variants in EEF2, which encodes the elongation factor 2 (eEF2), have been associated to Spinocerebellar ataxia 26 (SCA26) and more recently to a childhood-onset neurodevelopmental disorder with benign external hydrocephalus. The yeast model system used to investigate the functional consequences of the c.34 A > G EEF2 variant supported its pathogenicity by demonstrating it affects translational fidelity. In conclusion, the phenotype associated with the CASK variant is more severe and masks the milder phenotype of EEF2 variant.

Subdural Lesions Linking Additional Intracranial Spaces and Chronic Subdural Hematomas: A Narrative Review with Mutual Correlation and Possible Mechanisms behind High Recurrence.

The purpose of this study was two-fold. The first was to investigate the pathologic mechanisms underlying the formation of subdural fluid collection, an umbrella term referring to a condition commonly seen in the clinical setting. Accumulation of the cerebrospinal fluid (CSF) in the subdural space can be referred to in this disease category, disregarding the underlying source of the subdural fluid. However, in these two clinical situations, especially after trauma or brain surgery, fluid collection from the subarachnoid space (subdural hygroma) or from the ventricle to the subarachnoid space and infusion into the subdural space (external hydrocephalus), surgical management of critical patients may adopt the strategies of burr-hole, subduroperitoneal shunt, or ventriculoperitoneal shunt, which present distinctly different thoughts. Crucially, the former can be further transformed into chronic subdural hematoma (CSDH). The second significant theme was the pathogenesis of CSDH. Once the potential dural border cell (DBC) layer is separated such as if a wound is formed, the physiological mechanisms that seem to promote wound healing will resume in the subdural space as follows: coagulation, inflammation, fibroblast proliferation, neovascularization, and fibrinolysis. These aptly correspond to several key characteristics of CSDH formation such as the presence of both coagulation and fibrinolysis signals within the clot, neomembrane formation, angiogenesis, and recurrent bleeding, which contribute to CSDH failing to coagulate and absorb easily. Such a complexity of genesis and the possibility of arising from multiple pathological patterns provide a reasonable explanation for the high recurrence rate, even after surgery. Among the various complex and clinically challenging subdural lesions, namely, CSDH (confined to the subdural space alone), subdural hygroma (linked in two spaces), and external hydrocephalus (linked in three spaces), the ability to fully understand the different pathological mechanisms of each, differentiate them clinically, and devote more interventional strategies (including anti-inflammatory, anti-angiogenic, and anti-fibrinolysis) will be important themes in the future.

Successful surgical excision of congenital external hydrocephalus in a Telicherry kid: Case report

Successful surgical excision of congenital external hydrocephalus in a Telicherry kid: Case report

Peculiarities of brain structural changes in young patients with arterial hypertension

Actuality: Arterial hypertension (AH) is one of the most common diseases among diseases of the circulatory system and, together with other cardiovascular diseases, ranks first in the world and in Ukraine in terms of mortality rates. About 45% of patients do not know that they have hypertension. The scale of this problem in the world is evidenced by the fact that hypertension is found in every third adult. This disease occurs more often in the elderly, but its development often begins at a young age, and sometimes even in childhood. The intensive development of neuroimaging methods (CT and MRI) in recent decades makes it possible to detect various changes in the brain at early preclinical stages, which is of great importance for the implementation of adequate differentiated primary prevention of the development and progression of cerebrovascular disease and such unpleasant manifestations as cognitive deficits, dementia and ischemic stroke. The purpose of the study: to reveal the nature of structural changes in the brain in young patients with clinically confirmed hypertension. Material and methods: a study of structural changes in the brain was conducted based on the MRI examination data (1.5 T) of 54 young patients (18-44 years old) with hypertension of various degrees. Statistical analysis was carried out by the IBM SPSS Statistics 20 license program using the criterion of agreement with the test of static significance (Fisher's exact test -F). Results: Differences in brain structural changes were obtained in patients depending on the degree of hypertension. A significant difference according to the χ² criterion (р&lt;0.05) was found in the frequency of occurrence of mixed, external and internal hydrocephalus in patients with hypertension of the first and second century. Differences in the incidence of external and internal hydrocephalus in patients with AH I and AH III, AH II and AH III not detected by the χ² criterion (p&lt; 0.05). There are differences in the frequency of leukoaraiosis of the 0th, 1st, 2nd, and 3rd stages. according to the Fazekas scale between groups of patients with 1st stage hypertension, 2nd stage hypertension, and 3rd stage hypertension reliable according to the χ² criterion (р&lt; 0.05). Differences in the frequency of lacunar foci at different degrees of hypertension were not reliably detected. The analysis of the results of the assessment of structural changes of the brain by age showed that the main differences in the occurrence of mixed and external hydrocephalus are characteristic of all age groups. There is no difference in the frequency of internal hydrocephalus, mild and moderate leukoaraiosis in the groups of men and women. The frequency of occurrence of mixed, external hydrocephalus, the absence of leukoaraiosis and the presence of severe draining leukoaraiosis (in men, the severity of these changes prevails) is significantly different. No difference in the frequency of lacunar foci was found in the groups of men and women. Conclusions: Thus, hypertension is an age-dependent disease. No differences were found in the frequency of complaints of noise in the head, decreased attention and cognitive activity, sleep disturbances, and gender differences in the examined patients with hypertension of various degrees of severity. The main structural changes of the brain in the examined patients are various types of hydrocephalic manifestations, signs of leukoaraiosis (characteristic for the older age of 36-44 years) of one degree or another. Some gender specific features of brain structural changes in the examined patients were revealed (increased manifestations of hydrocephalus syndrome in men and absence of leukoaraiosis in them compared to women).

Transcranial optical monitoring for detecting intracranial pressure alterations in children with benign external hydrocephalus: a proof-of-concept study.

Benign external hydrocephalus (BEH) is considered a self-limiting pathology with a good prognosis. However, some children present a pathological intracranial pressure (ICP) characterized by quantitative and qualitative alterations (the so-called B-waves) that can lead to neurological sequelae. Our purpose was to evaluate whether there were cerebral hemodynamic changes associated with ICP B-waves that could be evaluated with noninvasive neuromonitoring. We recruited eleven patients (median age 16 months, range 7 to 55 months) with BEH and an unfavorable evolution requiring ICP monitoring. Bedside, nocturnal monitoring using near-infrared time-resolved and diffuse correlation spectroscopies synchronized to the clinical monitoring was performed. By focusing on the timing of different ICP patterns that were identified manually by clinicians, we detected significant tissue oxygen saturation ( ) changes ( ) and blood flow index (BFI) variability ( ) between regular and high-amplitude B-wave patterns. A blinded analysis looking for analogs of ICP patterns in BFI time traces achieved 90% sensitivity in identifying B-waves and 76% specificity in detecting the regular patterns. We revealed the presence of and BFI variations-detectable with optical techniques-during ICP B-waves in BEH children. Finally, the feasibility of detecting ICP B-waves in hemodynamic time traces obtained noninvasively was shown.

Clinical and molecular genetic findings of Crisponi/cold-induced sweating syndrome (CS/CISS) spectrum in patients from Turkey.

Crisponi/cold-induced sweating syndrome (CS/CISS) is a rare autosomal recessive disorder characterized by episodic hyperthermia, arthrogryposis, impaired feeding ability, and respiratory distress. The classic CS/CISS is mainly associated with CRLF1 and, rarely, CLCF1. PERCHING syndrome, previously known as CS/CISS type-3 associated with biallelic pathogenic variants in KLHL7, is notable for its few overlapping manifestations. This study presents genotype-phenotype relationships in CS/CISS-like spectrum associated with CRLF1 and KLHL7. Clinical findings of 19 patients from 14 families and four patients from three families were found in association with six different CRLF1 and three different KLHL7 variants, respectively. c.167T>C and c.713delC of the CRLF1 gene and the c.642G>C of the KLHL7 were novel. The c.708_709delCCinsT allele of CRLF1 was identified in 10 families from the Mardin province of Turkey, underlining that an ancestral haplotype has become widespread. CRLF1-associated phenotypes revealed novel manifestations such as prenatal oligohydramnios, benign external hydrocephalus, previously unreported dysmorphic features emerging with advancing age, severe palmoplantar keratoderma and facial erythema, hypopigmented macules and streaks, and recurrent cardiac arrests. KLHL7 variants presented with glabellar nevus flammeus, blepharophimosis, microcephaly, thin corpus callosum, and cleft palate. Abnormalities of sweating, observed in one patient reported herein, is known to be very rare among KLHL7-related phenotypes.

Clinical, psychological and neuroimaging characteristics of chronic tension headache in elderly persons

The aim of the study. To study clinical, psychological and neuroimaging characteristics of chronic tension headache (CTH) in the elderly.Materials and methods. 92 patients with CHTHN (37 men and 55 women aged 55–74 years) were examined. The control group consisted of 53 people (22 men and 31 women of the same age) who did not have neurological complaints, including headache. Pain intensity was assessed on a 10-point visual analogue scale. The severity of neurological symptoms was determined using the NIH-NINDS scale. For the study of cognitive functions, a short scale for assessing mental status was used Mini Mental State Examination (MMSE) and a frontal assessment battery (FAB). Memory was assessed according to the results of the memory subtest MMSE and the 10 words test. The level of attention was studied using the Schulte table, speech fluency – in the «fluency of speech» subtest of the FAB and in the test of verbal associations, visuospatial functions were assessed using the clock drawing test. Memory was assessed by the results of the subtest of memory MMSE and the 10-words test. The level of attention was studied using the Schulte table, the fluency of speech – in the subtest ‘fluency of speech’ FAB and in the verbal association test, visuospatial functions were assessed using the clock drawing test. Magnetic resonance imaging of the head was performed on a Magnetom Impact Expert (Siemens, Germany) tomograph with a magnetic field strength of 1.5 Tesla in T1 and T2 modes. The localization and severity of leukoaraiosis, signs of external and internal atrophy of the brain were determined, the linear dimensions of the anterior horns and central sections of the bodies of the lateral ventricles were measured, and the indices of the anterior horns and bodies of the lateral ventricles were calculated.Results. Patients with chronic tension headache were characterized by clinical and cognitive heterogeneity: frequent «non-standard» characteristics of headache, severe polymorphism of algic manifestations, high frequency of comorbid disorders, cognitive dysfunction and somatic burden, high representation of leukoaraiosis, external and internal hydrocephalus according to MRI study.

492 Development of Subdural Hematomas According to Size of the Subarachnoid Space in Benign External Hydrocephalus

INTRODUCTION: Benign External Hydrocephalus (BEH) is a common condition seen by pediatric neurologists and neurosurgeons in macrocephalic infants. It is accepted that patients with BEH are at increased risk of spontaneous subdural hematoma (SDHs) formation. METHODS: A large retrospective chart review was performed at a single institution for patients 2 years or younger with a diagnosis of BEH by neurology or neurosurgery and head circumference &gt;85th percentile. Demographic data, head circumference, and occurrence of subdural hematoma were extracted. MRIs were reviewed by two neuroradiologists and the SAS size measured. SAS size was graded per the Tucker et al grading system (&lt;5 mm width is grade 0, 5-9 mm is grade 1 and &gt; 10 mm is grade 2) for consistency. RESULTS: Over 2.5 million patients' charts were queried from a single institution and 480 patients met the inclusion criteria. Of that population, 187 children had an MRI of the brain available for review. The prevalence of spontaneous SDH in this BEH population over 25 years was 8.12% (39 patients of 480). The average size of the SAS was 5.86 mm (± 2.29 mm). A majority, 64.7%, had grade 1 SAS. About 39% had ventriculomegaly. There was no significant association between SAS grade and prevalence of SDHs (p = 0.124). SAS size was not significantly different between those with and without SDH (p= 0.402). CONCLUSION: BEH is a common condition seen by pediatric neurologists and neurosurgeons. Infants with BEH are thought to have increased risk of spontaneous SDH formation compared to the general population. A majority of infants had grade 1 SAS. The grade of SAS was not significantly associated with SDH formation and development of SDH was not associated with larger SAS size.

Severe phenotypes of B3GAT3-related disorder caused by two heterozygous variants: a case report and literature review

BackgroundLinkeropathies refers to a series of extremely rare hereditary connective tissue diseases affected by various glycosyltransferases in the biosynthesis of proteoglycans. We report for the first time two heterozygous variants of B3GAT3 in a Chinese infant, in whom Marfan syndrome was suspected at birth.Case presentationA 2-month-old boy from a non-consanguineous Chinese family without a family history presented severe phenotypes of joint dislocation, obvious flexion contractures of the elbow, arachnodactyly with slightly adducted thumbs, cranial dysplasia, foot abnormalities and aortic root dilation; Marfan syndrome was suspected at birth. Our patient was the youngest, at the age of 2 months, to experience aortic root dilation. Two B3GAT3 variants, NM_012200.2, c.752T>C, p.V251A and c.47C>A, p.S16*, with heterozygosity were identified in the patient by whole-exome sequencing; the variants were inherited from his parents. During close follow-up, significant changes in the cranial profile and obvious external hydrocephalus were present at the age of 7 months, which differs from previously reported cases.ConclusionWe diagnosed a patient with congenital heart defects at an early age with a B3GAT3-related disorder instead of Marfan syndrome and expanded the spectrum of B3GAT3-related disorders. We also provide a literature review of reported B3GAT3 cases; for at least one of the variants, this is the first report of genotype–phenotype correlations in individuals with cardiovascular defects being related to the acceptor substrate-binding subdomain of B3GAT3.

Corrigendum to the paper 'Re-evaluation of medical findings in alleged shaken baby syndrome and abusive head trauma in Norwegian courts fails to support abuse diagnoses'.

We recently carried out a retrospective evaluation of 17 child abuse cases in the Norwegian courts and concluded that 16 of those failed to support the evidence provided by expert witnesses of shaken baby syndrome.1 Our paper focussed on the possible mechanisms behind the children's intracranial injuries and unfortunately a couple of findings were missing or incompletely described. In case number 2, we described rib fractures with callus formation. This was true for some, but not all, of the fractures. We did not mention the term ‘flail chest’, which we have now found in the discharge record. This was not mentioned elsewhere on the medical chart, as a finding or medical condition. The same is true for the bilateral rather than unilateral retinal haemorrhages, which again only were described in the discharge records. In case number 6, we described clavicle and rib fractures, but unfortunately, we did not report that there was also a scapula fracture. Stray-Pedersen et al.2 have claimed that we made other errors. We can explain these by stating, as is also described in the article, that we did not have access to all the data at the time of the retrospective analysis. For example, we made a deliberate decision not to view any legal documents, in order to avoid introducing bias. These discrepancies included the fact that we did not mention the bilateral optic nerve sheath haematomas or metaphyseal fractures in case 4 because the medical charts did not contain this information. Furthermore, Stray-Pedersen et al. have stated that there was a rib fracture in case 4. This was suspected during the autopsy but could not be verified by microscopical examination. With regard to case 6, Stray-Pedersen et al stated that ‘the defendant confessed to having repeatedly shaken the child back and forth’. This information was not given in the article, as it was only mentioned in the legal documents and not in the medical records. However, following a reconstruction in the court, two medical experts, including Stray-Pedersen, concluded that the shaking that was demonstrated had insufficient power to cause intracranial findings. In addition, Stray-Pedersen et al. have disagreed with us on a few other cases, where we stand by our interpretations. For example, in case two, they claim that we have overlooked fresh haemorrhagic components in the subdural effusions. We have simply called these chronic subdural hematomas/hygromas, which we believe is reasonable since small rebledings in chronic SDHs are a common finding. Further, in case 4, which involved a one-month-old infant, Stray-Pedersen et al. claim that there was an acute subdural hematoma. We were unable to detect this on the images, it was not described in the medical charts, and we still find it hard to detect this finding. Our assessment of the low-quality computed tomography images for case 6 was that no definite acute, bilateral subdural haematoma was present, contrary to the view expressed by Stray-Pedersen et al. They also claim that the fractures described in case 6 were new. We felt that this was not the case, because callus was observed after another 11 days. In conclusion, we regret any inadvertent errors in our paper. The aim of our study was to analyse the intracranial findings presented by the court experts involved in these 17 cases and to evaluate if they showed evidence of traumatic origin per se. However, while it is important to present data as accurately and objectively as possible, it is also important to acknowledge the difficulties that arise in retrospective studies. We must stress that we are fully aware of the fact that child abuse exists, and of the importance of protecting abused infants. It is further not our conclusion that abuse can be excluded in all cases in this cohort. In the evaluation of these cases, it is self-evident that extracranial injuries such as bruises and different fractures must be taken into consideration. A comprehensive assessment of these was however beyond the scope of this article. That being said, we believe that the findings of our study still hold and that many of the intracranial findings in this cohort of possibly abused infants could be attributed to non-traumatic causes. One important example quoted in our paper was the group with benign external hydrocephalus. While Stray-Pedersen et al have made some valid points about our paper, it is interesting to note that they did not question our main intracranial findings.

External Hydrocephalus as a Cause of Infant Subdural Hematoma: Epidemiological and Radiological Investigations of Infants Suspected of Being Abused

BackgroundAcute subdural hematoma (ASDH) and chronic subdural hematoma (CSDH) in infants have been regarded as highly specific for abuse. Other causes of CSDH have not been investigated in a large population. PurposeThe purpose of this study was to investigate to what extent external hydrocephalus is present in infants with ASDH and CSDH undergoing evaluation for abuse. Material and methodsEighty-five infants suspected of being abused, with ASDH (n = 16) or CSDH (n = 69), were reviewed regarding age, risk factor profiles, craniocortical width (CCW), sinocortical width (SCW), frontal interhemispheric width (IHW), subarachnoid space width (SSW), and head circumference (HC). In infants with unilateral subdural hematoma (SDH), correlations between contralateral SSW and ipsilateral CCW and SDH width were investigated. ResultsInfants with CSDH had significantly lower mortality, were more often premature and male, and had significantly higher CCW, SCW, IHW, and SSW than infants with ASDH (P < 0.05). Ipsilateral CCW (R = 0.92, P < 0.001) and SDH width (R = 0.81, P < 0.01) correlated with contralateral SSW. Increased HC was more prevalent in infants with CSDH (71%) than in infants with ASDH (14%) (P < 0.01). Forty-two infants, all with CSDH, had at least one of CCW, SCW, or IHW ≥95th percentile. Twenty infants, all with CSDH, had CCW, SCW, and IHW >5 mm, in addition to increased HC. ConclusionA substantial proportion of infants with CSDH who had been suspected of being abused had findings suggesting external hydrocephalus.

Dural venous sinus anatomy in children with external hydrocephalus: analysis of a series of 97 patients

PurposeTo evaluate the anatomical variations of dural venous sinuses in children with external hydrocephalus, proposing a radiological grading of progressive anatomic restriction to venous outflow based on brain phase-contrast magnetic resonance venography (PC-MRV); to evaluate the correlation between positional plagiocephaly and dural sinuses patency; and to compare these findings with a control group to ascertain the role of anatomical restriction to venous outflow in the pathophysiology of external hydrocephalus.MethodsBrain MRI and PC MRV were performed in 97 children (76 males, 21 females) diagnosed with external hydrocephalus at an average age of 8.22 months. Reduction of patency of the dural sinuses was graded as 1 (stenosis), 2 (complete stop) and 3 (complete agenesis) for each transverse/sigmoid sinus and sagittal sinus. Anatomical restriction was graded for each patient from 0 (symmetric anatomy of patent dural sinuses) through 6 (bilateral agenesis of both transverse sinuses). Ventricular and subarachnoid spaces were measured above the intercommissural plane using segmentation software. Positional plagiocephaly (PP) and/or asymmetric tentorial insertion (ATI) was correlated with the presence and grading of venous sinus obstruction. These results were compared with a retrospective control group of 75 patients (35 males, 40 females).ResultsBoth the rate (84.53% vs 25.33%) and the grading (mean 2.59 vs mean 0.45) of anomalies of dural sinuses were significantly higher in case group than in control group. In the case group, sinus anomalies were asymmetric in 59 cases (right-left ratio 1/1) and symmetric in 22. A significant association was detected between the grading of venous drainage alterations and diagnosis of disease and between the severity of vascular anomalies and the widening of subarachnoid space (SAS). Postural plagiocephaly (39.1% vs 21.3%) and asymmetric tentorial insertion (35.4% vs 17.3%) were significantly more frequent in the case group than in the control group. When sinus anomalies occurred in plagiocephalic children, the obstruction grading was significantly higher on the flattened side (p ≤ 0.001).ConclusionDecreased patency of the dural sinuses and consequent increased venous outflow resistance may play a role in the pathophysiology of external hydrocephalus in the first 3 years of life. In plagiocephalic children, calvarial flattening may impact on the homolateral dural sinus patency, with a possible effect on the anatomy of dural sinuses and venous drainage in the first months of life.

Re-evaluation of abusive head trauma in Norway appears flawed.

A review by Wester et al in Acta Paediatrica presented 17 adjudicated cases of Norwegian infants with abusive head trauma (AHT).(1) Their interpretation of the medical findings identified alternative diagnoses for 16 of the 17 children and suggested that the injuries were not inflicted and may have been the result of spontaneously occurring events. The authors stated that eight children had clinical and radiological characteristics consistent with external hydrocephalus, complicated by chronic subdural haematoma, and that six had findings compatible with hypoxic-ischaemic insults. Fractures were attributed to prematurity and sequelae from birth trauma. If their interpretations are correct, this study calls for a thorough review of how the medical community, forensic experts and the courts manage AHT cases. However, there are several elements of concern in the Wester et al study that we believe need clarification before a conclusion can be reached.

Imaging differentiation on extra-axial fluid collections: a clinical Image.

A 3 month – old male patient with a history of seizures presents to the Pediatric neurosurgery department with his parents, on initial evaluation a head circumference of 47.5 cm (&gt; percentile 99 on the WHO chart) was seen. A brain MRI was performed and an extra axial fluid collections (Chronic subdural hematoma and benign external hydrocephalus) was diagnosed. His parents refer a normal delivery without any complication and the medical history and records denied any previous trauma. A neurosurgical evacuation of the subdural hematoma was performed, and a complete improvement of his seizure was seen on 3 months follow up. Benign external hydrocephalus (BEH) has been proposed as a risk factor for the presence of chronic subdural hematoma (SDH) in infants (1). The SDH formation in the presence of BEH has been reported to be a venous rupture either spontaneously or following minor trauma from the bridging veins traversing the subdural/subarachnoid space (Red arrow , image D ) that are stretched with enlarged extra?axial collections (2). Epidemiologically there are striking similarities between these entities (3) that can be differentiated by some MRI findings.The signs that help us in the differentiation are: the subarachnoid layer visible (yellow arrow image B, showing a displaced subarachnoid layer downward by a fluid between it and the dura) the absence of the cortical vein sign and the differential on fluid intensity on T1-T2 weighted images (Images A,B,C,D) (4). Surgery evacuation is necessary for the patients with chronic subdural hematoma associated with BEH and neurological signs of increased intracranial pressure like macrocrania, seizures and altered level of consciousness (Image F , showing the subdural space without hematoma and the subarachnoid layer downward it ) (5).

Re-evaluation of medical findings in alleged shaken baby syndrome and abusive head trauma in Norwegian courts fails to support abuse diagnoses.

The criteria for diagnosing abusive head trauma (AHT) are not well defined and this condition might be diagnosed on failing premises. Our aim was to review criminal AHT cases in Norwegian courts by scrutinising the underlying medical documentation. Cases were identified in the data registry for Norwegian courts from 2004 to 2015. Documentation was obtained from relevant health institutions. The medical co-authors first made independent evaluations of the documentation for each child, followed by a consensus evaluation. A total of 17 children (11 boys) were identified, all diagnosed as AHT by court appointed experts, 15 were infants (mean age 2.6months). A high proportion (41.2%) was born to immigrant parents and 31.3% were premature. The medical findings could be explained by alternative diagnoses in 16 of the 17 children; 8 boys (7 infants - mean age 2.9months) had clinical and radiological characteristics compatible with external hydrocephalus complicated by chronic subdural haematoma. Six children (five infants with mean age 2.1months) had a female preponderance and findings compatible with hypoxic ischaemic insults. The medical condition in most children had not necessarily been caused by shaking or direct impact, as was originally concluded by the court experts.