Literature DB >> 27932436

Multifactorial pathological hip subluxation in neurofibromatosis type-1 (NF1) due to intra-articular plexiform neurofibroma, lumbar radiculopathy and neurofibromatous polyneuropathy.

Waqar Waheed1, Diego F Diego F Lemos2, Nelms Nathaniel Nelms3, Rup Tandan4.   

Abstract

Neurofibromatosis type-1 (NF1) is a multisystem disorder with very rare descriptions of hip instability. We report a case of a 37-year-old man with known NF1 and childhood-onset of left foot drop, who developed persistent left hip pain following a minor trauma. Physical examination revealed left-sided mild foot drop, hip abductor weakness, bilateral sensory loss in feet and an antalgic gait. Work-up revealed anterolateral subluxation of the left femoral head along with left hip plexiform neurofibroma (PN), dysplastic and degenerative changes, neurofibromatous neuropathy and chronic left L5 radiculopathy. Initial improvement after surgical resection of the PN was unsustained and followed by increasing pain and recurrent hip subluxation, prompting a total hip arthroplasty which resulted in marked improvement of symptoms. Our patient, unlike those in other reports, illustrates multiple aetiologies of hip dislocation in NF1 including local factors, lumbar radiculopathy and polyneuropathy, reinforcing the importance of a multidisciplinary approach in the management of such cases. 2016 BMJ Publishing Group Ltd.

Entities:  

Mesh:

Year:  2016        PMID: 27932436      PMCID: PMC5174849          DOI: 10.1136/bcr-2016-217971

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  21 in total

1.  Aberrant regulation of ras proteins in malignant tumour cells from type 1 neurofibromatosis patients.

Authors:  T N Basu; D H Gutmann; J A Fletcher; T W Glover; F S Collins; J Downward
Journal:  Nature       Date:  1992-04-23       Impact factor: 49.962

2.  Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits.

Authors:  Rosa Nguyen; Lan Kluwe; Carsten Fuensterer; Michael Kentsch; Reinhard Edgar Friedrich; Victor-Felix Mautner
Journal:  J Pediatr       Date:  2011-05-31       Impact factor: 4.406

3.  Association between benign and malignant peripheral nerve sheath tumors in NF1.

Authors:  T Tucker; P Wolkenstein; J Revuz; J Zeller; J M Friedman
Journal:  Neurology       Date:  2005-07-26       Impact factor: 9.910

4.  Elevated risk for MPNST in NF1 microdeletion patients.

Authors:  T De Raedt; H Brems; P Wolkenstein; D Vidaud; S Pilotti; F Perrone; V Mautner; S Frahm; R Sciot; E Legius
Journal:  Am J Hum Genet       Date:  2003-03-26       Impact factor: 11.025

5.  Neurofibromatous neuropathy in neurofibromatosis 1 (NF1).

Authors:  R E Ferner; R A C Hughes; S M Hall; M Upadhyaya; M R Johnson
Journal:  J Med Genet       Date:  2004-11       Impact factor: 6.318

6.  Recurrent spontaneous hip dislocation in a patient with neurofibromatosis type 1: a case report.

Authors:  John G Galbraith; Joseph S Butler; James A Harty
Journal:  J Med Case Rep       Date:  2011-03-16

7.  Imatinib mesylate for plexiform neurofibromas in patients with neurofibromatosis type 1: a phase 2 trial.

Authors:  Kent A Robertson; Grzegorz Nalepa; Feng-Chun Yang; Daniel C Bowers; Chang Y Ho; Gary D Hutchins; James M Croop; Terry A Vik; Scott C Denne; Luis F Parada; Cynthia M Hingtgen; Laurence E Walsh; Menggang Yu; Kamnesh R Pradhan; Mary K Edwards-Brown; Mervyn D Cohen; James W Fletcher; Jeffrey B Travers; Karl W Staser; Melissa W Lee; Marcie R Sherman; Cynthia J Davis; Lucy C Miller; David A Ingram; D Wade Clapp
Journal:  Lancet Oncol       Date:  2012-10-23       Impact factor: 41.316

8.  Comparative effectiveness of 18F-FDG PET/CT versus whole-body MRI for detection of malignant peripheral nerve sheath tumors in neurofibromatosis type 1.

Authors:  Thorsten Derlin; Katharina Tornquist; Silvia Münster; Ivayla Apostolova; Christian Hagel; Reinhard E Friedrich; Ulrike Wedegärtner; Victor F Mautner
Journal:  Clin Nucl Med       Date:  2013-01       Impact factor: 7.794

9.  Characteristics of children enrolled in treatment trials for NF1-related plexiform neurofibromas.

Authors:  A Kim; A Gillespie; E Dombi; A Goodwin; W Goodspeed; E Fox; F M Balis; B C Widemann
Journal:  Neurology       Date:  2009-10-20       Impact factor: 9.910

10.  Pathological dislocation of the hip in neurofibromatosis: a case report.

Authors:  Manuele Lampasi; Tiziana Greggi; Alessandra Sudanese
Journal:  Chir Organi Mov       Date:  2008-05-21
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