scholarly journals Bone Lesions in Children with Neurofibromatosis

2021 ◽  
Author(s):  
Nikolaos Laliotis

Neurofibromatosis is often related with severe orthopaedic disorders in children. Bone lesions are rare but pose severe difficulties in management. It affects the spine and long bones. Lesions are associated either from enlargement of neurofibromas that affect the normal growth or from primary neurofibromatosis of long bones. Dystrophic scoliosis appears with short curves, with kyphosis and rotation of the apical vertebrae. Usually affect the thoracic spine, with penciling of the ribs. Surgical treatment is challenging in cases of rapid progression. Scoliosis may appear with curvatures similar to those in idiopathic scoliosis, without dysplastic changes of the vertebrae. Anterior bowing of the tibia is manifestation of NF and is distinguished from the benign posterolateral bowing. Evaluation of the medullary canal and presence of cystic lesions in the tibia is essential. Progression to pseudoarthrosis or pathologic fracture is common. Surgical management of tibial pseudoarthrosis remains a difficult procedure. Pseudoarthrosis may appear in fibula, radius or ulna but are extremely rare. Irregular eccentric bone cysts in long bones that are commonly diagnosed after a pathologic fracture, must be differentiated for NF. Malignant transformation of neurofibromas must be considered when there is rapid progression of the lesion.

1998 ◽  
Vol 11 (01) ◽  
pp. 08-18 ◽  
Author(s):  
C. W. McIlwraith ◽  
J. A. Auer ◽  
Brigitte von Rechenberg

SummaryCases of cystic bone lesions in horses and humans were reviewed in the literature. These lesions are radiolucent areas of bone, recognized as subchondral cystic lesions in the horse (SCL), intra-osseous ganglia (IOG), subchondral bone cysts secondary to osteoarthrosis (OAC), and unicameral bone cysts (UCB) in humans. Their morphology is quite similar, consisting of lesions with a distinct cyst wall, and a cavity filled with fibrous tissue and yellowish mucoid fluid. The lesions are surrounded by sclerotic bone and can be easily diagnosed radiographically. SCL, IOG and OAC occur in the subchondral bone close to the adjacent joint, whereas UCB occur in the metaphysis of long bones. Their aetiology and pathogenesis is still unknown, although primary damage to the subchondral bone, cartilage or local blood supply and growth disturbances are discussed. In this review 703 lesions of SCL in horses, 289 lesions of IOG and 1460 lesions of UCB in humans were compared in their anatomical location and clinical signs. SCL and OAC resembled each other with respect to anatomical location. A correlation of affected bones could not be found for all four groups. Clinical presentation concerning age was most similar for SCL and UCB with both lesions mainly occurring in young individuals. Gender predominance of males was present in SCL, IOG and UCB. Clinical diagnosis was either incidental, or connected with intermittent pain in all lesions except for OAC. Additionally, the lesions were also found in conjunction with degenerative joint disease (SCL, OAC) or pathological fractures (UCB). Cystic bone lesions were either treated conservatively, surgically with curettage alone, curettage in combination with grafting procedures, or intra-lesional application of corticosteroids. SCL and UCB were similar in their biological behaviour concerning their slow response to the therapy and relatively high recurrence rate. None of the cystic bone lesions were comparable, and a common aetiology and pathogenesis could not be found.In a literature review cases of cystic bone lesions in horses and humans were compared with the goal to find a common aetiology and pathogenesis. Cystic bone lesions occur in horses as subchondral cystic lesions (SCL), and in humans as either intra-osseous ganglia (IOG), subchondral cystic lesions secondary to osteoarthrosis (OAC) or unicameral bone cysts (UCB). IOG and OAC compare with SCL mainly in the anatomical location. IOG and SCL resemble each other in size, clinical signs and histology, whereas UCB and SCL show a similar biological behaviour regarding their therapeutic response and recurrence rate. None of the cystic bone lesions in humans were comparable to the SCL in horses in all aspects. A common aetiology and pathogenesis could not be established.


2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Nikolaos G. Lasanianos ◽  
Ioannis Spanos ◽  
Aggeliki Papaioannou ◽  
Elisavet Paneri

Simple bone cysts are nonsymptomatic lesions. They typically involve the medullary cavity, but they can also be found in nonlong bones such as the calcaneum. Their treatment remains controversial varying from observation and conservative healing to irritating injections or bone grafting. In the case of a pathologic fracture, surgical treatment seems most appropriate especially when the cyst is situated on a weight-bearing bone. We present herein the rare case of a spontaneously healed pathological fracture over a critical-size calcaneal cyst of a patient reluctant to undergo surgical treatment. An interpretation of the healing procedure as well as a review of the literature is presented.


2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Murat Arıkan ◽  
Ahmet Yıldırım ◽  
Güray Togral ◽  
Alp Burak Ekmekçi

Nasu-Hakola disease, which is also known as polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), is a rare and mortal human genetic disorder (Verloes et al., (1997) and Bianchin et al., (2004)). Nasu-Hakola is a progressive disease characterized by early onset cognitive dementia and bone cysts (both evident by the third decade). The disease has a worldwide distribution, but most patients have been reported in Finland and in Japan (Montalbetti et al., (2004)). In the literature less than 200 cases are reported and only a few of them are about the surgical treatment for the extremity (Madry et al., (2007)). Most patients die by their fourth or fifth decade because of neurologic problems. Surgeons generally prefer conservative treatment modalities in the treatment of cystic lesions of the bone in this syndrome. In this case report, we presented a 42-year-old male with Nasu-Hakola disease having bilateral painful talar lipomembranous cystic lesions treated with curettage and iliac bone grafting. He is in the 3rd year of his followup after surgery and he has not any extremity complaints, but his neurological problems sustain. Our aim in this study is to show the beneficial aspect of surgical intervention in the cystic lesions of Nasu Hakola disease in the skeleton to obtain the patient a painless joint although surgery is rarely performed in this systemic and progressive disease.


2021 ◽  
pp. 65-68
Author(s):  
Vikash M. Harinandan ◽  
Ragini Kumari ◽  
Kunal Shankar ◽  
Laljee Chaudhary ◽  
Debarshi Jana

Objective: Many different neoplastic and non-neoplastic lesions involve the skeletal system. Clinical and radiological tools primarily assess the nature of these lesions. The aim of this study was to analyze the cystic bone lesions in a pathologic point of view. Methods:All bone cysts evaluated under the guidance of clinical information and radiological images. Descriptive data such as age, gender, tumor site, symptoms, and clinical and radiological ndings obtained from the hospital's database system. Results: There were 96 cystic bone lesions; 47 were aneurysmal bone cysts (ABCs), 37 were simple bone cysts (SBCs), one was a lesion with features of both ABC and SBC, four were intraosseous ganglia, four were epidermoid cysts, and three were hydatid cysts. The mean ages of the patients with ABCs and SBCs were 18.7 ± 12.8 years (range, 3-75 years) and 23.8 ± 13.3 years (range, 3-62 years), respectively. Most of the lesions located in the long bones. Conclusions: Cystic lesions of the bone rarely encountered in daily pathology routine. As with all conditions affecting the skeletal system, one of the most important steps towards an accurate pathological diagnosis is to perform with clinical and radiological information while evaluating the patients.


2020 ◽  
Vol 101 (5) ◽  
pp. 685-690
Author(s):  
Jun Wang ◽  
N V Kharchenko ◽  
V Y Karpenko

Currently, with the development of the concept of cancer treatment, the survival rate of patients has increased significantly, but the percentage of the frequency of various metastatic lesions remains high. The bones of the skeleton are one of the main parts of metastases. In cancer patients, bone metastases usually mean that the disease is at an advanced stage, and the prognosis is not good. These patients often suffer from many complications, including pain, decreased mobility, pathological fractures, etc. The quality of life of patients is seriously deteriorating. Therefore, the main goal of surgical treatment of patients with bone metastases is the earliest possible restoration the function of the affected limb, pain relief, prevention of pathological fractures and the improvement of the quality of life of patients after surgery. This literature review analyzes the incidence of bone metastases in patients, and the dependence of metastatic lesions of long tubular bones on localization incidence of bone metastases in different body parts. The domestic and foreign literature on the surgical treatment of patients with metastatic bone lesions was analyzed. The results of the analysis show that the features of surgical treatment are becoming the main factors influencing the prognosis in patients with metastatic lesions of the long bones.


2007 ◽  
Vol 8 (1) ◽  
pp. 11-15 ◽  
Author(s):  
A. Megaro ◽  
S. Marchesi ◽  
U. E. Pazzaglia

2021 ◽  
Vol 19 (3) ◽  
pp. 144-151
Author(s):  
P. E. ELDZAROV ◽  

The work is devoted to improving the effectiveness of treatment of patients with complications and consequences of fractures of the long bones of the extremities by improving and developing new surgical techniques aimed at early individual social and household rehabilitation due to the maximally complete and rapid restoration of the integrity and functions of the damaged segment. Reconstructive operations were performed in 285 patients with delayed fracture consolidation, incorrectly fused fractures, false joints, and false joints with chronic osteomyelitis. The analysis of the applied treatment methods effectiveness from the viewpoint of optimizing the treatment process allowed us to develop an algorithm for the surgical treatment of patients with complications and consequences of fractures of the long bones of the extremities. The use of the proposed algorithm in surgical treatment maximally eliminates possible errors and increases the treatment effectiveness.


Hand ◽  
2016 ◽  
Vol 12 (5) ◽  
pp. NP55-NP57
Author(s):  
Eduardo P. Zancolli ◽  
John M. Ranson ◽  
Sunil M. Thirkannad

Background: Aneurysmal bone cysts are an uncommon form of benign primary bone tumors; affection of the hand is very rare. Methods: A rigorous review of the literature showed that this type of tumor has not previously been described in the pisiform. In this article, we report the occurrence of an aneurysmal bone tumor in the pisiform of the left hand of a 19-year-old male. Results: This tumor was successfully treated through surgical excision with an uneventful recovery. We describe the process behind the diagnosis of the lesion and the subsequent treatment in an attempt to highlight the rare but possible occurrence of aneurysmal bone cysts in the pisiform. Conclusion: Appropriate treatment of aneurysmal bone cysts in this location is required, due to the anatomical nature of the pisiform itself with the risks of pathologic fracture and ulnar nerve compression.


2004 ◽  
Vol 11 (2) ◽  
pp. 69-73
Author(s):  
A P Bergaliev ◽  
A P Pozdeev ◽  
A N Bergaliev ◽  
A P Pozdeev

Study of blood circulation intensity and bone tissue functional activity was performed in 81 children bone cysts (including 10 children with cyst recurrence) using polyphasic scintigraphy. Most typical picture in presence or absence of pathologic fracture as well as in pathologic recurrence is described. Interrelation between the fracture and rate of cyst recurrence is detected. Scintigraphic criterion for prognosing of solitary bone cyst recurrence is suggested.


1997 ◽  
Vol 106 (9) ◽  
pp. 729-732 ◽  
Author(s):  
Daniel R. Seely ◽  
George A. Gates

Parosteal osteogenic sarcoma (POS) is an uncommon surface bone tumor, most often arising from the metaphyseal end of long bones. Involvement of the cranial bones is rare, with only 1 case of mastoid bone POS previously reported in the literature. Two patients with POS of the mastoid are presented, 1 followed up for 25 years after surgical treatment. The presenting signs and symptoms, as well as distinctive radiographic findings, are discussed. Histologic features are also described. Typically, cranial POS appears as a sessile, densely ossified surface growth with radiating bone spicules that blend with surrounding soft tissue. Treatment is en bloc resection, which is curative in most cases.


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