scholarly journals An Illustrative Case of Neurofibromatosis Type 1 and NF1 Microdeletion

2010 ◽  
Vol 1 (3) ◽  
pp. 133-135 ◽  
Author(s):  
L.A. Praxedes ◽  
F.M. Pereira ◽  
J.F. Mazzeu ◽  
S.S. Costa ◽  
D.R. Bertola ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Illustrative Case

scholarly journals Surgical repair of a large intrathoracic meningocele associated with neurofibromatosis type 1 after failed cystoperitoneal shunts: illustrative case

2021 ◽  
Vol 2 (10) ◽  
Author(s):  
Christopher H. F. Sum ◽  
Lai-Fung Li ◽  
Benedict B. T. Taw ◽  
Wai-Man Lui ◽  
Ko-Yung Sit ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Muscle Flap ◽  
Neurofibromatosis Type ◽  
Definitive Treatment ◽  
Illustrative Case ◽  
Thoracoscopic Approach ◽  
Regional Flap ◽  
Definitive Repair ◽  
Intrathoracic Meningocele

BACKGROUND Surgical treatment of intrathoracic meningoceles, commonly associated with neurofibromatosis type 1 (NF1), aims to reduce sac size for symptomatic relief. The procedures can be divided into cerebrospinal fluid diversion and definitive repair. The authors describe the management of an intrathoracic meningocele in a 56-year-old female with preexisting NF1. OBSERVATIONS The patient presented with progressive dyspnea. Magnetic resonance imaging revealed a left hemithoracic meningocele arising from the thecal sac at C7–T2. Two attempts at diversion by cystoperitoneal shunts resulted in recurrence. For definitive repair, T2–3 costotransversectomy was performed, and intradural closure of the meningocele opening was performed utilizing spinal dura and autologous fascia lata graft. Trapezius muscle regional flap was turned for reinforcement. Persistent leak warranted reoperation 7 days later. A transthoracic approach was undertaken using video-assisted thoracoscopic resection of the sac at aortic arch level, with reinforcement by latissimus dorsi flap and synthetic materials. Mechanical pleurodesis was performed. Intradural repair of the meningocele opening was revised. LESSONS Inherent dural abnormality makes repair difficult for meningoceles associated with NF1. A combined intradural and thoracoscopic approach with regional muscle flap and synthetic material reinforcement is a unique method for definitive treatment. Some essential points of perioperative management are highlighted.

scholarly journals The recurrent plexiform neurofibroma of the scalp in neurofibromatosis type 1: illustrative case

2021 ◽  
Vol 1 (2) ◽  
Author(s):  
Galih Indra Permana ◽  
M. Arifin Parenrengi ◽  
Wihasto Suryaningtyas ◽  
Dyah Fauziah ◽  
Muhammad Azzam
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Peripheral Nerves ◽  
Symptomatic Relief ◽  
Operative Procedure ◽  
Plexiform Neurofibroma ◽  
Tumor Resection ◽  
Neurofibromatosis Type ◽  
Illustrative Case ◽  
Unusual Variant

BACKGROUNDPlexiform neurofibroma is a benign tumor of the peripheral nerves. It is an unusual variant of neurofibroma originating from all parts of the nerve. Plexiform neurofibroma is primarily pathognomonic and exhibits an unusual variant from neurofibromatosis type 1 (NF1). The possibility of malignancy and recurrence are the main reasons for long-term, close follow-up.OBSERVATIONSThe authors report a case of a 14-year-old girl with a recurrent plexiform neurofibroma derived from the peripheral nerves, which also presented with a typical sign of NF1 disease. The aim of the tumor resection is symptomatic relief.LESSONSAccomplishing a good outcome can be related to good perioperative planning and a precise operative procedure. The result of anatomical pathology determines the prognosis of the patient. Clinical examination and radiological studies are needed to evaluate the recurrence of complications after surgical procedures.

Social information processing of children with Neurofibromatosis Type 1

2008 ◽  
Author(s):  
Jonathan M. Kurss ◽  
Anna E. Craig ◽  
Jennifer Reiter-Purtill ◽  
Kathryn Vannatta ◽  
Cynthia Gerhardt
Keyword(s):  
Information Processing ◽  
Neurofibromatosis Type 1 ◽  
Social Information Processing ◽  
Social Information ◽  
Neurofibromatosis Type

Lovastatin improves impaired LTP-like plasticity in patients with Neurofibromatosis Type 1

2011 ◽  
Vol 42 (01) ◽  
Author(s):  
F. Mainberger ◽  
N. Jung ◽  
M. Zenker ◽  
I. Delvendahl ◽  
U. Wahlländer ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type

Intima-Type Vasculopathy in Neurofibromatosis Type 1

2014 ◽  
Vol 45 (S 01) ◽  
Author(s):  
J. Keppler ◽  
A. Fiedler
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type
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