Primitive megalencephaly in children: Natural history, medium term prognosis with special reference to external hydrocephalus

Laubscher, Bernard; Deonna, Thierry; Uské, A; Melle, Guy van

doi:10.1007/bf01959405

Cited by 54 publications

(61 citation statements)

References 13 publications

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“…Several older case series of BESS mention examples of small subdural collections of CSF, blood, or a mixture of these 2 in asymptomatic children. 2,3,5,10,13 Because of small numbers and variable methods of ascertainment, no credible estimate of the prevalence of subdural collections among infants with BESS can be developed from this older literature. Nor can these cases be cleared retrospectively of the suspicion of unrecognized abuse.…”

Section: Discussionmentioning

confidence: 99%

Macrocephaly in infancy: benign enlargement of the subarachnoid spaces and subdural collections

Tucker

Choudhary²,

Piatt

2016

PED

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OBJECTIVE Benign enlargement of the subarachnoid spaces (BESS) is a common finding on imaging studies indicated by macrocephaly in infancy. This finding has been associated with the presence of subdural fluid collections that are sometimes construed as suggestive of abusive head injury. The prevalence of BESS among infants with macrocephaly and the prevalence of subdural collections among infants with BESS are both poorly defined. The goal of this study was to determine the relative frequencies of BESS, hydrocephalus, and subdural collections in a large consecutive series of imaging studies performed for macrocephaly and to determine the prevalence of subdural fluid collections among patients with BESS. METHODS A text search of radiology requisitions identified studies performed for macrocephaly in patients ≤ 2 years of age. Studies of patients with hydrocephalus or acute trauma were excluded. Studies that demonstrated hydrocephalus or chronic subdural hematoma not previously recognized but responsible for macrocephaly were noted but not investigated further. The remaining studies were reviewed for the presence of incidental subdural collections and for measurement of the depth of the subarachnoid space. A 3-point scale was used to grade BESS: Grade 0, < 5 mm; Grade 1, 5–9 mm; and Grade 2, ≥ 10 mm. RESULTS After exclusions, there were 538 studies, including 7 cases of hydrocephalus (1.3%) and 1 large, bilateral chronic subdural hematoma (0.2%). There were incidental subdural collections in 21 cases (3.9%). Two hundred sixty-five studies (49.2%) exhibited Grade 1 BESS, and 46 studies (8.6%) exhibited Grade 2 BESS. The prevalence of incidental subdural collections among studies with BESS was 18 of 311 (5.8%). The presence of BESS was associated with a greater prevalence of subdural collections, and higher grades of BESS were associated with increasing prevalence of subdural collections. After controlling for imaging modality, the odds ratio of the association of BESS with subdural collections was 3.68 (95% CI 1.12–12.1, p = 0.0115). There was no association of race, sex, or insurance status with subdural collections. Patients with BESS had larger head circumference Z-scores, but there was no association of head circumference or age with subdural collections. Interrater reliability in the diagnosis and grading of BESS was only fair. CONCLUSIONS The current study confirms the association of BESS with incidental subdural collections and suggests that greater depth of the subarachnoid space is associated with increased prevalence of such collections. These observations support the theory that infants with BESS have a predisposition to subdural collections on an anatomical basis. Incidental subdural collections in the setting of BESS are not necessarily indicative of abusive head injury.

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Section: Discussionmentioning

confidence: 99%

Macrocephaly in infancy: benign enlargement of the subarachnoid spaces and subdural collections

Tucker

Choudhary²,

Piatt

2016

PED

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“…We could find only 12 previously reported spontaneous, nontraumatic SDC in children with prior imaging documentation. 7,20,26,[31][32][33] The best documented of these reports is from Amodio et al, 20 who described a 3.5-month-old, former 32-week preterm boy, with grade 1 germinal matrix hemorrhage presenting with macrocrania. Initial head ultrasound examination demonstrated enlarged extra-axial spaces but no SDC.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of Subdural Collections in Children with Macrocrania

Greiner

Richards

Caré

et al. 2013

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE:The relationship between enlarged subarachnoid spaces and subdural collections is poorly understood and creates challenges for clinicians investigating the etiology of subdural collections. The purpose of this study was to determine the prevalence of subdural collections on cross sectional imaging in children with macrocephaly correlating with subarachnoid space enlargement.

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“…Therefore, neurosurgical treatment is generally not undertaken for uncomplicated BEH. The etiology of BEH remains poorly understood, but children are often macrocephalic, which may be a familial condition [5, 10]. …”

Section: Introductionmentioning

confidence: 99%

A Theoretical Model of Benign External Hydrocephalus That Predicts a Predisposition towards Extra-Axial Hemorrhage after Minor Head Trauma

Papasian

Frim²

2000

Pediatr Neurosurg

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Introduction: There is controversy over whether there exists a predisposition towards bleeding into the subdural space in infants with benign external hydrocephalus (BEH) or other enlargement of the extra-axial space (e.g. subdural hygroma). The presumed etiology implicates shear forces in over-stretching the extra-axial blood vessels. We have created a model of the intracranial space that approximates certain aspects of BEH. Using this model, we predict situations where children with BEH will bleed into the extra-axial space when normal infants will not. Methods: The cranial model consists of two spheres representing the brain and the skull. The distance between them represents the width of the extra-axial space. The spheres are concentric (with interspheric distance equal to N) in the normal condition and nonconcentric in BEH. In BEH, the distance between the two spheres varies from N to Q (0 < N ≤ Q) over a 906 M ≤ Q, with the translation orthogonal to the vector of N and parallel to that of M or vice versa), then the final length V_f of a vein V is V_f = (N² + M)^1/2, and the stretch ratio V_f/V_i is: V_f/V_i = (N² + M²)^1/2/N·S_i, with S_i the slack factor, where i represents either n for normal or b for BEH and M = N in the normal condition. Results: Given an equivalent capacity of veins to resist stretch injury (based on the proportion of change in length), for brain translations after a low-impact head injury, stretch ratios for BEH veins range from 1.677 to 3.436, whereas in the normal condition they range from 1.061 to 1.179. Therefore, for an increase in subarachnoid space from 3 (normal) to 6 mm (BEH), brain translocation in BEH will stretch veins beyond an average breaking point when the translation for the normal condition will not. Conclusions: Mathematical modeling of the cranial vault produces a relationship between venous stretch and the width of the extra-axial space. These equations predict an increased frequency of venous stretch injury in the situation of widened extra-axial space. Such venous injury is consistent with forces generated by minor trauma. This relationship, as predicted by our model, could underlie a predisposition towards extra-axial bleeding after minor head trauma in infants with BEH.

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Primitive megalencephaly in children: Natural history, medium term prognosis with special reference to external hydrocephalus

Cited by 54 publications

References 13 publications

Macrocephaly in infancy: benign enlargement of the subarachnoid spaces and subdural collections

Macrocephaly in infancy: benign enlargement of the subarachnoid spaces and subdural collections

Prevalence of Subdural Collections in Children with Macrocrania

A Theoretical Model of Benign External Hydrocephalus That Predicts a Predisposition towards Extra-Axial Hemorrhage after Minor Head Trauma

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