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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 photograph showing skin lesions - café au lait spots (black arrows) and axillary freckling (white arrow)
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Figure 1: Clinical photograph showing skin lesions - café au lait spots (black arrows) and axillary freckling (white arrow)

Mentions: A 16-year-old boy presented with the complaints of pain in the left hip associated with the inability to bear weight following a trivial fall. On examination, the affected limb was 1.5 cm short, abducted and externally rotated. Joint line was tender and attempted movements were painful. On general examination, patient had 8 café au lait spots over the body, bilateral axillary freckles and multiple palpable neurofibromas in the subcutaneous tissues of forearm, thighs and back [Figure 1]. Patient met three out of seven criteria described for the diagnosis of NF-1 [Table 1].2 Plain radiograph and computed tomography scan of pelvis revealed an ill-defined lytic lesion causing pathological fracture - dislocation of the left hip [Figure 2]. Magnetic resonance imaging (MRI) showed additional soft tissue involvement and joint effusion [Figure 3]. MRI picture was in favor of a giant cell tumor. Ultrasound guided fine-needle aspiration cytology showed scanty cellularity with round to oval cells having minimal pleomorphism; hyperchromatic nucleus and moderate cytoplasm with spindle cells and osteoblasts. These features were suggestive of a sarcomatous lesion.


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 photograph showing skin lesions - café au lait spots (black arrows) and axillary freckling (white arrow)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Clinical photograph showing skin lesions - café au lait spots (black arrows) and axillary freckling (white arrow)
Mentions: A 16-year-old boy presented with the complaints of pain in the left hip associated with the inability to bear weight following a trivial fall. On examination, the affected limb was 1.5 cm short, abducted and externally rotated. Joint line was tender and attempted movements were painful. On general examination, patient had 8 café au lait spots over the body, bilateral axillary freckles and multiple palpable neurofibromas in the subcutaneous tissues of forearm, thighs and back [Figure 1]. Patient met three out of seven criteria described for the diagnosis of NF-1 [Table 1].2 Plain radiograph and computed tomography scan of pelvis revealed an ill-defined lytic lesion causing pathological fracture - dislocation of the left hip [Figure 2]. Magnetic resonance imaging (MRI) showed additional soft tissue involvement and joint effusion [Figure 3]. MRI picture was in favor of a giant cell tumor. Ultrasound guided fine-needle aspiration cytology showed scanty cellularity with round to oval cells having minimal pleomorphism; hyperchromatic nucleus and moderate cytoplasm with spindle cells and osteoblasts. These features were suggestive of a sarcomatous lesion.

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