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Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
Skeletal abnormalities occur in 68%: Fibrous dysplasia of the skull.Scoliosis.Unilateral hypoplasia of a variety of bony structures.
Robotic-Assisted Resection of Fibrous Dysplasia of the Ribs
Published in Wickii T. Vigneswaran, Thoracic Surgery, 2019
Hiroko Nakahama, Wickii T. Vigneswaran
Fibrous dysplasia comprises approximately 30%–50% of benign bone tumors [1]. Fibrous dysplasia can be divided into two types: monostotic with one bone involvement and polyostotic with multiple bone involvement. Monostotic fibrous dysplasia occurs in approximately 70%–80% of cases and arises most commonly in the ribs, proximal femur, tibia, and skull [1,7]. Polyostotic fibrous dysplasia involves more than one bone and is closely associated with McCune-Albright syndrome [1]. Fibrous dysplasia arises sporadically and affects the ribs in approximately 6%–20% of cases [8]. Surgical resection is indicated when lesions become symptomatic and cause significant deformation, or when malignant disease is in question. Malignant degeneration occurs in approximately 0.5%–4% of cases [8–10].
Abdominal mass: Fibrous dysplasia
Published in Alisa McQueen, S. Margaret Paik, Pediatric Emergency Medicine: Illustrated Clinical Cases, 2018
This patient has fibrous dysplasia. Fibrous dysplasia is a bone disease that replaces normal bone with a fibrous-type tissue (as seen in the left upper quadrant on these images). This tissue is not as hard as normal bone and therefore more susceptible to fracture. One or multiple bones may be affected, most commonly the femur, tibia, or humerus. However, any bone may be affected. Fibrous dysplasia usually occurs between 3 and 15 years of age. Fibrous dysplasia is also a feature in McCune-Albright syndrome, a genetic disorder affecting bone, skin, and endocrine systems.
Multiple primary intraosseous meningioma mimicking fibrous dysplasia: a case report
Published in British Journal of Neurosurgery, 2023
Büşra Yaprak Bayrak, Çiğdem Vural, İsa Çam, Demir Kürşat Yildiz, Seda Duman Öztürk, Gür Akansel, Emine Binnaz Sarper
A 49-year-old female with premenstrual headache presented with two bony protuberances, a larger one located in the right frontoparietal bone and smaller one located in left parietal bone. Computed tomography (CT) revealed intradiploic diffuse sclerotic lesions measuring 5 × 4.5 × 1.5 cm at the right frontoparietal region and 2.5 × 1.0 × 2.5 cm at the left parietal region expanding the calvaria without bony destruction. MRI showed the lesions to be non-enhancing and hypointense on T1 and T2-weighted sequences (Figure 1(A)). She also had plane x-rays and whole body bone scintigraphy (WBBS). The results led to a suspected diagnosis of fibrous dysplasia. Repeated MRI at 25 months follow-up showed the enlargement of both lesions: measuring 7 × 7×2 cm on the right and 3.5 × 1.5 × 3.5cm on the left. Other radiological findings were similar to previous MRI (Figure 1(B–D)). She remained intact neurologically but complained with premenstrual headaches and asymmetric skull appearance. Thus, an operation was performed to remove the right hyperostotic bone and related durameter. The left lesion was not operated due to lack of clinical symptoms and remains under follow-up.
Frontal osteoplastic flap without frontal sinus obliteration for orbital roof decompression
Published in Orbit, 2021
Matthew Kim, Marc Otten, Michael Kazim, David A. Gudis
A 20-year-old male presented to the emergency department with several months of progressive right ptosis and diplopia, particularly on gaze elevation. Additionally, he endorsed several weeks of forehead swelling along with a recent history of acute mononucleosis. On exam, he had right ptosis with mild restriction of eye elevation, as well as right hypoglobus and fullness to palpation of the globe. Visual acuity was normal, with no relative afferent pupillary defect, and visual fields were full to confrontation. Fundoscopic exam revealed a normal optic nerve with presence of choroidal folds. Computed tomography and magnetic resonance imaging demonstrated a massive fibro-osseous lesion encompassing the entire frontal bone with bilateral orbital and ethmoid extension, as well as a right 2.1 cm superomedial orbital mucocele with mass effect on the right superior rectus and globe (Figure 1). Based on these findings, the patient underwent right anterior orbitotomy with excision of mucocele. Bone biopsy obtained at the time of surgery revealed no abnormal bone or evidence of fibrous dysplasia. The patient’s diplopia resolved after the procedure, but serial imaging revealed interval growth of the fibro-osseous lesion with intermittent right orbital swelling and pressure as well as progressive orbital asymmetry over the following 2 years. Following multidisciplinary evaluation, surgery was ultimately recommended.
First radiobiological characterization of the McCune–Albright syndrome: influence of the ATM protein and effect of statins + bisphosphonates treatment
Published in International Journal of Radiation Biology, 2021
Jean-Thomas Bachelet, Adeline Granzotto, Mélanie Ferlazzo, Laurène Sonzogni, Elise Berthel, Clément Devic, Nicolas Foray
The diagnosis of MAS is usually made in childhood and from clinical features only rather from genetic studies. The initial medical care is generally based on endocrine management to fight against precocious puberty. The number and size of fibrous dysplasia lesions are unpredictable and a regular monitoring is required. As a result, the management of fibrous dysplasia lesions is mainly performed at the adult age. Currently, surgical management has become the gold-standard treatment but alternatives including radiotherapy are still discussed (Bousson et al. 2014; Chapurlat and Orcel 2008). Besides, while spontaneous malignant transformation is rare for MAS patients (between 0.5 and 5% of cases), the major risk linked to radiotherapy may be radiation-induced malignant transformations, such as osteosarcoma, fibrosarcoma and chondrosarcoma (Chapurlat and Meunier 2000; Collins et al. 2003; Kaushik et al. 2002; Ozek et al. 2002; Qu et al. 2015; Ruggieri et al. 1994; Ruiz-Godoy et al. 1999; Yabut et al. 1988). Furthermore, some tissue complications of radiotherapy have been reported among the surgeons community but no systematic survey has been conducted yet (Chapurlat and Meunier 2000) (Bachelet, personal communication). In parallel to surgery, bisphosphonates, proposed to increase bone density, were shown to improve the MAS patient outcome (Benhamou et al. 2016; Corsi et al. 2017; Lala et al. 2006; Majoor et al. 2017; O'Sullivan and Zacharin 2002).