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Benign enlargement of the subarachnoid space in infancy

Benign enlargement of the subarachnoid spaces in infancy (BESS or BESSI) also known as benign external hydrocephalus (BEH), is as the name implies, a benign enlargement of subarachnoid spaces seen in infancy. It usually involves the frontal lobe subarachnoid spaces and it is characterized clinically by macrocephaly or frontal bossing.
 

Epidemiology

  • commonest cause of macrocephaly aka macrocrania 9
  • more commonly in boys than in girls
  • in the majority of cases, there is a family history of macrocephal

Clinical presentation

Often discovered incidentally or when patients undergo neuroimaging in the workup of macrocephaly. These patients are usually neurologically normal and have no history of trauma.

Pathophysiology

The aetiology is not clear. It has been described as a variation of normal development of the brain, whereby there is a transient accumulation of cerebrospinal fluid in the frontal region. It is also thought to be due to the delayed development or delayed function of the arachnoid villi at the sagittal sinus.

Radiographic features

Ultrasound, CT and MRI imaging of the brain may all demonstrate the characteristic findings seen in BESS:

  • widening of the bifrontal and anterior interhemispheric CSF spaces
    • no consensus of cut-off values exists 5
    • findings should be correlated with patient age 5,7:
      • an estimated equation for both inter hemispheric width (IHW), craniocortical width CCW and sinocortical width SCW has been suggested 7
      • IHW >= 5mm in NEONATES 
      • IHW >8.5mm in 1y old
  • no flattening of adjacent gyri
  • usually normal sulci posteriorly
  • the anterior fontanelle is frequently enlarged with the enlargement of the subarachnoid space in the frontoparietal regions
  • normal ventricular size, no pressure effects on the surrounding brain tissue, no cerebral atrophy
  • no blood products on MRI study
  • another key distinction between benign enlargement of the subarachnoid spaces and a subdural fluid collection is that in the former the cortical veins will be adjacent to the inner table of the calvarium on MR and ultrasound; whereas in the latter the veins are displaced away from the inner table, as the arachnoid membrane and subarachnoid space are displaced.

Complications

While these findings are benign in many cases, there is an increased risk of subdural haemorrhage, either spontaneously or following minor trauma. Subdural hematoma in a patient with BESS should not be interpretated as suggestive of non-accidental injury without other stigmata 4

A low percentage of patients may develop communicating hydrocephalus, which may warrant treatment.

Treatment and prognosis

The condition resolves spontaneously by the age of 2 years. Although the macrocephaly may persist, the subarachnoid space fluid collection will resolve or become minimal as the child grows older.

Studies show that infants with macrocephaly or rapid head-growth, CT findings of enlarged subarachnoid spaces, normal-to-minimally increased ventricular size and who have a parent with macrocephaly, have a good developmental prognosis and a characteristic pattern of neuromotor development in the first year.

A review of literature has described that although most children with external hydrocephalus do well, a substantial number show temporary or permanent psychomotor delay. 

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