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Pathological fracture dislocation of the acetabulum in a patient with neurofibromatosis-1.

Saibaba B, Sen RK, Sharma M, Nahar U - Indian J Orthop (2016 Jan-Feb)

Bottom Line: NF affecting the pelvic girdle is extremely rare.Pathological fracture of the acetabulum leading to anterior hip dislocation in a patient with NF-1 has never been reported in the literature.The paper presents the clinical symptomatology, the course of management and the successful outcome of such a rare case of NF-1.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

ABSTRACT
Skeletal neurofibromatosis (NF) commonly manifests as scoliosis and tibial dysplasias. NF affecting the pelvic girdle is extremely rare. Pathological fracture of the acetabulum leading to anterior hip dislocation in a patient with NF-1 has never been reported in the literature. The paper presents the clinical symptomatology, the course of management and the successful outcome of such a rare case of NF-1. Histopathological and immunohistochemistry studies showing abundant spindle cells, which are S-100 positive and of neural origin are the classical hallmarks of neurofibromatous lesions. Tumor resection and iliofemoral arthrodesis can be considered as a valid option in young patients with pathological fracture dislocation of the acetabulum.

No MeSH data available.


Related in: MedlinePlus

Clinical photographs showing functional outcome at 1-year followup
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Figure 7: Clinical photographs showing functional outcome at 1-year followup

Mentions: Histopathology revealed dense collagenous tissue cores with spindle cells having blunt nuclei with minimal atypia and no mitosis or necrosis [Figure 6a]. On immunohistochemistry, cells were S-100 positive and of neural origin [Figure 6b]. These findings were consistent with neurofibroma. The postoperative period was uneventful. Partial weight bearing was allowed at 6 weeks and full weight bearing at 10 weeks. At 1-year followup, the patient was comfortable, pain free, able to ambulate unassisted, stand on one limb, sit and climb stairs without any difficulty [Figure 7].


Pathological fracture dislocation of the acetabulum in a patient with neurofibromatosis-1.

Saibaba B, Sen RK, Sharma M, Nahar U - Indian J Orthop (2016 Jan-Feb)

Clinical photographs showing functional outcome at 1-year followup
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4759865&req=5

Figure 7: Clinical photographs showing functional outcome at 1-year followup
Mentions: Histopathology revealed dense collagenous tissue cores with spindle cells having blunt nuclei with minimal atypia and no mitosis or necrosis [Figure 6a]. On immunohistochemistry, cells were S-100 positive and of neural origin [Figure 6b]. These findings were consistent with neurofibroma. The postoperative period was uneventful. Partial weight bearing was allowed at 6 weeks and full weight bearing at 10 weeks. At 1-year followup, the patient was comfortable, pain free, able to ambulate unassisted, stand on one limb, sit and climb stairs without any difficulty [Figure 7].

Bottom Line: NF affecting the pelvic girdle is extremely rare.Pathological fracture of the acetabulum leading to anterior hip dislocation in a patient with NF-1 has never been reported in the literature.The paper presents the clinical symptomatology, the course of management and the successful outcome of such a rare case of NF-1.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

ABSTRACT
Skeletal neurofibromatosis (NF) commonly manifests as scoliosis and tibial dysplasias. NF affecting the pelvic girdle is extremely rare. Pathological fracture of the acetabulum leading to anterior hip dislocation in a patient with NF-1 has never been reported in the literature. The paper presents the clinical symptomatology, the course of management and the successful outcome of such a rare case of NF-1. Histopathological and immunohistochemistry studies showing abundant spindle cells, which are S-100 positive and of neural origin are the classical hallmarks of neurofibromatous lesions. Tumor resection and iliofemoral arthrodesis can be considered as a valid option in young patients with pathological fracture dislocation of the acetabulum.

No MeSH data available.


Related in: MedlinePlus