Altered cerebrospinal fluid dynamics in neurofibromatosis type l: severe arachnoid thickening in patients with neurofibromatosis type 1 may cause abnormal CSF dynamic.
BACKGROUND
The object of this study is to understand abnormal dynamic of cerebrospinal fluid (CSF) in patients with neurofibromatosis type 1 (NF1), which may cause temporal lobe herniation and bulging of temporal fossa.


METHODS
Four patients, three females and one male, with NF1 were studied retrospectively. They presented with a similar craniofacial deformity, which consisted of pulsatile exophthalmos, an enlarged bony orbit, dysplasia of the sphenoid wing with the presence of a herniation of the temporal lobe into the orbit, and a bulging temporal fossa.


CONCLUSIONS
Surgical exploration demonstrated abnormally thickened arachnoid membrane in one case. Protruding temporal lobe, which was one of the main symptoms in NF1 patients, could be stopped by control of intracranial pressure (ICP) via programmable ventriculoperitoneal shunt (VPS) or extra ventricle drainage implantation. The dense fibrosis of the arachnoid membrane and consequent altered hemispheric CSF dynamics may cause symptoms including pulsatile exophthalmos and consequent worsening of vision, prolapse of the temporal lobe, and enlargement of the temporal fossa. This finding may not present with general features of hydrocephalus, so that delays in diagnosis often result.


CONCLUSIONS
For the NF1 patients with cranio-orbito-temporal deformities, prior to any surgical reconstruction, control of increased ICP (IICP) should be primarily considered.
Division of Pediatric Neurosurgery, Charité Universitätsmedizin, Berlin, Germany.|Department of Neurosurgery, Universitätsmedizin, Mainz, Germany.
Young Sill
YS
Kang
Craniofacial Reformation Clinic, Department of Neurosurgery, Brain Korea 21 Project for Medical Science, Yonsei University College of Medicine, Seoul, South Korea.|Pediatric Neurosurgery, Department of Neurosurgery, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, South Korea.
Eun-Kyung
EK
Park
Craniofacial Reformation Clinic, Department of Neurosurgery, Brain Korea 21 Project for Medical Science, Yonsei University College of Medicine, Seoul, South Korea.|Department of Plastic Surgery, Craniofacial Reformation Clinic, Yonsei University College of Medicine, Seoul, South Korea.
Yong-Oock
YO
Kim
Craniofacial Reformation Clinic, Department of Neurosurgery, Brain Korea 21 Project for Medical Science, Yonsei University College of Medicine, Seoul, South Korea.|Pediatric Neurosurgery, Department of Neurosurgery, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, South Korea.
Ju-Seong
JS
Kim
Craniofacial Reformation Clinic, Department of Neurosurgery, Brain Korea 21 Project for Medical Science, Yonsei University College of Medicine, Seoul, South Korea.|Pediatric Neurosurgery, Department of Neurosurgery, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, South Korea.
Dong-Seok
DS
Kim
Division of Pediatric Neurosurgery, Charité Universitätsmedizin, Berlin, Germany.
U W
UW
Thomale
Craniofacial Reformation Clinic, Department of Neurosurgery, Brain Korea 21 Project for Medical Science, Yonsei University College of Medicine, Seoul, South Korea. shimkyuwon@yuhs.ac.|Pediatric Neurosurgery, Department of Neurosurgery, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, South Korea. shimkyuwon@yuhs.ac.
Kyu-Won
KW
Shim
D000293:Adolescent; D000328:Adult; D001099:Arachnoid / Q000000981:diagnostic imaging* / Q000601:surgery; D015170:Cerebrospinal Fluid Pressure / Q000502:physiology*; D002648:Child; D005260:Female; D006801:Humans; D057446:Hydrodynamics; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013702:Temporal Lobe / Q000000981:diagnostic imaging* / Q000601:surgery
Arachnoidal thickening; Increased intracranial pressure (IICP); Neurofibromatosis type 1 (NF1); Orbito-temporal deformity; Sphenoid dysplasia