Benign enlargement of the subarachnoid space in infancy
Benign enlargement of the subarachnoid spaces (BESS), 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.
- more commonly in boys than in girl
- in the majority of cases, there is a family history of macrocephal
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.
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.
Ultrasound, CT and MRI imaging of the brain may demonstrate findings seen in BESS:
- widening of the bifrontal and anterior interhemispheric CSF spaces (>5mm)
- 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
- it is associated with normal ventricular size. There are no pressure effects on the surrounding brain tissue or 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
While these findings are benign in many cases, there is an increased risk of subdural haemorrhage, either spontaneously or following minor trauma. 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.
- 1. Nickel RE, Gallenstein JS. Developmental prognosis for infants with benign enlargement of the subarachnoid spaces. Dev Med Child Neurol. 1987;29 (2): 181-6. - Pubmed citation
- 2. Suara RO, Trouth AJ, Collins M. Benign subarachnoid space enlargement of infancy. J Natl Med Assoc. 2001;93 (2): 70-3. - Free text at pubmed - Pubmed citation
- 3. Zahl SM, Egge A, Helseth E, Wester K. Benign external hydrocephalus: a review, with emphasis on management. Neurosurg Rev. 2011; 34(4): 417-432. - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Benign subdural effusions of infancy||✓|
|Benign macrocephaly of infancy||✓|
|Benign subarachmoid space enlargement||✗|
|Benign external hydrocephalus of infancy||✓|