Hydrocephalus in Patients with Neurofibromatosis Type 1: MR Imaging Findings and the Outcome of Endoscopic Third Ventriculostomy

✓ For benign intrinsic tectal tumors causing triventricular obstructive hydrocephalus, cerebrospinal fluid diversion followed by neuroimaging is a widely accepted treatment plan. In this report, the authors describe two children with focal enhancing tectal lesions that caused acute, symptomatic hydrocephalus. One child had neurofibromatosis Type 1 (NF1). In both children the hydrocephalus was effectively treated by endoscopic third ventriculostomy. Following this procedure, serial imaging studies revealed not only that the ventriculomegaly had resolved, but also that the enhancing tectal tumors had regressed and disappeared over time. The time to complete involution of these tumors was 18 months for the child with NF1 and 12 months for the other child. To the authors' knowledge, this is the first report of the involution of enhancing tectal tumors after endoscopic third ventriculostomy. The possible mechanisms for this unexpected result are discussed.

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Unilateral absence of sphenoid wing in a Neurofibromatosis type 1 patient: imaging findings

Journal of Clinical and Experimental Investigations ◽

10.5799/ahinjs.01.2013.03.0303 ◽

2013 ◽

Vol 4 (3) ◽

Author(s):

Arzu Canan ◽

Emine Dağıstan ◽

Abdullah Yüksel Barut ◽

Yeşim Karagöz

Keyword(s):

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Imaging Findings

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Whole body MR imaging in neurofibromatosis type 1

European Journal of Radiology ◽

10.1016/j.ejrad.2008.10.024 ◽

2009 ◽

Vol 69 (2) ◽

pp. 236-242 ◽

Cited By ~ 23

Author(s):

S.F.L. Van Meerbeeck ◽

K.L. Verstraete ◽

S. Janssens ◽

G. Mortier

Keyword(s):

Mr Imaging ◽

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Whole Body

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Serial MR imaging and 1H-MR spectroscopy of unidentified bright objects in a case of neurofibromatosis type 1

Brain and Development ◽

10.1016/j.braindev.2005.02.008 ◽

2005 ◽

Vol 27 (8) ◽

pp. 595-597 ◽

Cited By ~ 8

Author(s):

Atsushi Imamura ◽

Naoki Matsuo ◽

Miho Okuda ◽

Hideyuki Morita ◽

Masako Iwata ◽

...

Keyword(s):

Mr Imaging ◽

Neurofibromatosis Type 1 ◽

Mr Spectroscopy ◽

Neurofibromatosis Type ◽

1H Mr Spectroscopy ◽

Unidentified Bright Objects

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Neurofibromatosis-associated nerve sheath tumors

Neurosurgical FOCUS ◽

10.3171/foc.2006.20.1.2 ◽

2006 ◽

Vol 20 (1) ◽

pp. 1-10 ◽

Cited By ~ 9

Author(s):

Judith A. Murovic ◽

Daniel H. Kim ◽

David G. Kline

Keyword(s):

Diagnostic Criteria ◽

Current Literature ◽

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Imaging Findings ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Examination Protocol ◽

Nerve Sheath

In this paper the authors describe a patient with neurofibromatosis Type 1 (NF1) who presented with sequelae of this disease. They also review the current literature on NF1 and NF2 published between 2001 and 2005. The method used to obtain information for the case report consisted of a family member interview and a review of the patient's chart. For the literature review the authors used the search engine Ovid Medline to identify papers published on the topic between 2001 and 2005. Neurofibromatosis Type 1 appears in approximately one in 2500 to 4000 births, is caused by a defect on 17q11.2, and results in neurofibromin inactivation. The authors reviewed the current literature with regard to the following aspects of this disease: 1) diagnostic criteria for NF1; 2) criteria for other NF1-associated manifestations; 3) malignant peripheral nerve sheath tumors (PNSTs); 4) the examination protocol for a patient with an NF1-related NST; 5) imaging findings in patients with NF1; 6) other diagnostic studies; 7) surgical and adjuvant treatment for NSTs and malignant PNSTs; and 8) hormone receptors in NF1-related tumors. Pertinent illustrations are included. Neurofibromatosis Type 2 occurs much less frequently than NF1, that is, in one in 33,000 births. Mutations in NF2 occur on 22q12 and result in inactivation of the tumor suppressor merlin. The following data on this disease are presented: 1) diagnostic criteria for NF2; 2) criteria for other NF2 manifestations; 3) malignant PNSTs in patients with NF2; 4) examination protocol for the patient with NF2 who has an NST; and 5) imaging findings in patients with NF2. Relevant illustrations are included. It is important that neurosurgeons be aware of the sequelae of NF1 and NF2, because they may be called on to treat these conditions.

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