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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

Intraoperative photographs showing (a) Skin incision (b) deformed femoral head (c) 14 hole DCP (d) iliofemoral arthrodesis
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Figure 4: Intraoperative photographs showing (a) Skin incision (b) deformed femoral head (c) 14 hole DCP (d) iliofemoral arthrodesis

Mentions: A wide local excision followed by arthrodesis of the joint was planned. Considering the extent of bony and soft tissue involvement, we used a modification of the ilioinguinal and iliofemoral approach to have a wide exposure. We used the conventional ilioinguinal incision and combined it with femoral part of the iliofemoral incision [Figure 4a]. On exposing the pelvis, anatomy was distorted. The deformed femur head and acetabulum with deficient pubic rami were visualized [Figure 4b]. There was extensive soft tissue involvement adjacent to the acetabulum and lower part of the ilium. Femur osteotomy at the level of lesser trochanter was done. The entire acetabulum with 2-3 cm clear margin of the ilium was resected along with the abnormal soft tissue. Iliofemoral arthrodesis was done using a 14 hole stainless steel dynamic compression plate [Figures 4c–d, and 5].


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)

Intraoperative photographs showing (a) Skin incision (b) deformed femoral head (c) 14 hole DCP (d) iliofemoral arthrodesis
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 4: Intraoperative photographs showing (a) Skin incision (b) deformed femoral head (c) 14 hole DCP (d) iliofemoral arthrodesis
Mentions: A wide local excision followed by arthrodesis of the joint was planned. Considering the extent of bony and soft tissue involvement, we used a modification of the ilioinguinal and iliofemoral approach to have a wide exposure. We used the conventional ilioinguinal incision and combined it with femoral part of the iliofemoral incision [Figure 4a]. On exposing the pelvis, anatomy was distorted. The deformed femur head and acetabulum with deficient pubic rami were visualized [Figure 4b]. There was extensive soft tissue involvement adjacent to the acetabulum and lower part of the ilium. Femur osteotomy at the level of lesser trochanter was done. The entire acetabulum with 2-3 cm clear margin of the ilium was resected along with the abnormal soft tissue. Iliofemoral arthrodesis was done using a 14 hole stainless steel dynamic compression plate [Figures 4c–d, and 5].

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