Craniofacial Fibrous Dysplasia: Experience at San José Hospital, Bogotá, Colombia.

INTRODUCTION: Fibrous dysplasia is a disorder in which normal bone is gradually replaced by immature fibro-osseous tissue, with an incidence of less than 7% of all benign bone tumors. The management of this disease is a challenge for plastic surgeons and neurosurgeons. GOAL: To describe the diagnostic, therapeutic, and outcome approach of patients with craniofacial fibrous dysplasia seen at the Plastic Surgery Service of the Hospital San José in Bogotá, Colombia. METHODS: This is a descriptive and retrospective case series study of patients diagnosed with monostotic and polyostotic fibrous dysplasia treated at the Plastic Surgery Department of Hospital San José during the period from January 1, 2010, to July 31, 2023. RESULTS: All (n=10) of the patients had monostotic craniofacial fibrous dysplasia. The most affected bones in patients with monostotic fibrous dysplasia were zone I bones (n=10, 100%), followed by zone II bones (n=2, 20%). Patients with zone I and II involvement manifested throbbing headaches associated with phosphenes and tinnitus (n=8, 80%) and pain during occlusion associated with edema in the affected cheek (n=5, 50%). Physical examination showed that patients with orbital wall involvement (zone I bone) had ocular dystopia (n=7, 70%).Regarding the treatment received by the patients, 90% (n=9) of the patients received surgical management as primary treatment, with orbitotomy, replacement, and/or remodeling of the roof and lateral wall of the orbit with bone graft, drilling, canthoplasty, ciliary suspension being the most frequently performed procedure (n=6, 60%). Of the patients, 20% (n=2) required reintervention. CONCLUSIONS: FD is a slowly progressive benign fibro-osseous disease that requires a timely, individualized, and multidisciplinary diagnosis and treatment to obtain favorable clinical and surgical results.The mainstay of treatment is surgery as a preventive measure since it is important to avoid future functional alterations that, depending on the location of the dysplasia, would cause a high risk of alteration of adjacent structures.

Fibrous dysplasia limited to an ossicle.

Fibrous dysplasia is an unusual pathologic condition caused by abnormal bone metabolism. Temporal bone involvement is often seen, but it is uncommon to find fibrous dysplasia limited to the middle ear, especially originating in and confined to a single ossicle. Here we report a case of osteofibrous dysplasia limited exclusively to an ossicle (malleus) causing gradual conductive hearing loss, which recovered after eventual complete removal of the dysplastic area. The lesion showed firm attachment to adjacent structures and initial removal was not possible. This report provides information to help other otologic surgeons facing similar conditions.

Kyphotic deformity of the lumbar spine due to a monostotic fibrous dysplasia of the second lumbar vertebra: a case report and its surgical management.

Monostotic fibrous dysplasia (MFD) of the lumbar spine represents an exceedingly rare lesion. A 26-year-old patient presented with a progressive osteolytic lesion of the vertebral body L2 and the diagnosis of MFD. A minimally invasive left-sided eXtreme Lateral Interbody Fusion (XLIF) approach with resection of the vertebral body L2 with placement of a mesh cage was performed. No complications were observed perioperatively and the symptoms rapidly improved. Minimally invasive piecemeal resection with a combined dorsolateral approach showed a favorable clinical and radiological outcome and seems to be a safe and reliable technique for MFD.

The surgical management of monostotic fibrous dysplasia of the inferior turbinate.

Fibrous dysplasia is a non-neoplastic, sporadic, slowly progressing disease of the bone in which normal bone is replaced by abnormally overgrowing lesions. There are three different types of fibrous dysplasia: monostotic (affecting a single bone), poliostotic (affecting multiple bones) and syndromic, when it is associated with other diseases (such as McCune Albright syndrome). Fibrous dysplasia affects cranio-facial bones in 10% of the cases. However, its occurrence in the inferior turbinate is extremely rare. To the best of our knowledge, only four cases of monostotic form have been reported so far. Hereafter we describe a case of monostotic FD of the inferior turbinate surgically treated with a trans-nasal endoscopic partial maxillectomy type II.

Monostotic fibrous dysplasia at C7 treated with vertebroplasty: a case report and review of the literature.

BACKGROUND: Monostotic fibrous dysplasia (MFD) involving the spine is rare, and the treatment options are controversial. Surgery is needed when patients suffer from persistent pain, spinal cord compression/injury, and vertebral collapse/instability. Treatment methods include biopsy/observation, corpectomy with instrumented fusion, posterior fusion, vertebroplasty (VP), curettage and bone graft, and complete removal of the vertebra with a combined anterior and posterior fusion procedure. CASE PRESENTATION: The patient was a 56-year-old woman with a 2-year history of neck pain. No obvious abnormalities were detected on neurological or physical examination, and laboratory findings were all within normal limits. An imaging examination suggested a C7 vertebral bone tumor. The patient refused to continue conservative observation treatment and requested surgery. Open VP of the C7 vertebral body was carried out, and her postoperative neck pain was completely relieved. The postoperative pathological results supported the diagnosis of fibrous dysplasia, and the patient was ultimately diagnosed with MFD. At the 12-month follow-up visit, the patient reported no clinical symptoms, and no signs of tumor recurrence were detected. CONCLUSION: VP can relieve pain while stabilizing the spine. Thus, the surgical treatment of MFD vertebral lesions by VP is a valuable option.

Serie de Casos Clínicos: Displasia Osteofibrosa Craneofacial. Resolución Quirúrgica / Case Series: Craniofacial Fibrous Dysplasia. Surgical Treatment

INTRODUCCIÓN: La Displasia Fibrosa Ósea Craneofacial es una lesión ósea benigna en la que se da una sustitución de tejido óseo normal por tejido fibro-óseo. Desarrollada a partir de una mutación genética. Subordinada en variantes: monostósica y polistósica. Escasos casos son reportados sobre malignización de la patología. El Diagnóstico se realiza con la sospecha clínica y se confirma mediante exámenes de imagen y anatomopatológicos. CASO CLÍNICO: El presente trabajo presenta una serie de tres casos diagnosticados de Displasia Osteofibrosa Craneofacial en el Hospital José Carrasco Arteaga y Hospital del Río, Cuenca-Ecuador, en los que por el cuadro clínico de los pacientes se decidió tratamiento quirúrgico. EVOLUCIÓN: En todos los casos, la evolución fue favorable. Se logró resultados positivos, reducción de síntomas y mejoría estética general en todos los casos. CONCLUSIÓN: Se obtienen mejores resultados con los tratamientos quirúrgicos actuales (remodelado más congelamiento óseo), entre ellos: buenos resultados estéticos, mejor calidad de vida, menor riesgo de infecciones. En el presente trabajo los tres participantes sometidos a resolución quirúrgica señalaron que el tratamiento reflejó positivamente en el ámbito funcional, estético y emocional. (au)

BACKGROUND: Craniofacial Bone Fibrous Dysplasia is a benign bone lesion where normal bone tissue is replaced with fibrous tissue. Developed from a genetic mutation. Subordinated in variants: monostotic and polyistotic. Few cases are reported on malignancy of the pathology. The diagnosis is determined with clinical suspicion and confirmed by imaging and pathology tests. CASE REPORT: In this paper, we present a series of three cases diagnosed with Craniofacial Fibrous Dysplasia at José Carrasco Arteaga Hospital and Hospital del Río, Cuenca-Ecuador. All of patients were treated with surgery, because of the clinical features. EVOLUTION: In all the cases, the patient evolution was favorable. Reduction of symptoms and general aesthetic improvement were achieved in all cases. CONCLUSION: Better results are obtained with the current surgical treatments (bone remodeling plus bone freezing), among them: good aesthetic results, better quality of life, lower risk of infections. In the present paper, the three patients treated with the surgical procedure indicated that the treatment reflected positively in the functional, aesthetic and emotional aspects.(au)

A Rare Case of Radiculopathy: Monostotic Fibrous Dysplasia of the Sacrum.

Fibrous dysplasia is a bone disease characterized by an osteoblastic dysfunction resulting in a fibrous replacement of the normal medullary bone. We describe the case of a 33-year-old who presented with low back pain irradiating to her right leg. Both the computed tomography scan and magnetic resonance imaging showed an osteolytic, multicystic lesion of the right hemi-sacrum with invasion of the right S1 foramen. She underwent foraminotomy and curettage of the lesion. Histological diagnosis was fibrous dysplasia, without features of malignant transformation. Three years after surgery the patient is asymptomatic and imaging is stable. This is the fifth known case of monostotic fibrous dysplasia involving the sacrum, a rare entity that must be considered in the differential diagnosis when approaching patients with sacral lesions.

A displasia fibrosa é uma doença óssea caracterizada por uma disfunção osteoblástica que resulta na substituição do osso medular normal por tecido fibroso. Descrevemos o caso de uma doente de 33 anos que se apresentou com lombalgia e ciatalgia direita. A tomografia computorizada e ressonância magnética lombares revelaram uma lesão osteolítica e multiquística no hemissacro direito com invasão do foramen de S1. A doente foi submetida a foraminotomia e curetagem da lesão. O diagnóstico histológico foi de displasia fibrosa, sem sinais de transformação maligna. Três anos após a cirurgia, a doente está assintomática e os estudos de imagem apresentam estabilidade da lesão. Este é o quinto caso conhecido na literatura de displasia fibrosa monostótica com atingimento do sacro, uma entidade rara que deve ser considerada no diagnóstico diferencial de doentes com lesões do sacro.

Surgical Treatment of Polyostotic Craniomaxillofacial Fibrous Dysplasia Associated With Acromegalia: The McCune - Albright Syndrome.

INTRODUCTION: The McCune-Albright syndrome (MAS) is a complex congenital disorder caused by the embryonic post-zygotic somatic activating mutations in the GNAS1 gene. In such syndrome, phenotypes are heterogeneous and comprised polyostotic/monostotic fibrous dysplasia, café au lait macules, and hyperfunctioning endocrinopathies as the excess growth hormone. Likewise, acromegaly, as a manifestation of the endocrine hyperfunction, is unusual and affects about 20% of patients with MAS. CASE PRESENTATION: This research study describes a case of a 31-year-old female subject presenting polyostotic fibrous dysplasia with severe facial involvement, along with acromegaly and the MAS. The case was satisfactorily managed by surgical re-alignment and presented no clinical signs of relapse in a 12-year follow-up period. Finally, a literature review was conducted to discuss the standard protocols and the controversies when treating such cases. CONCLUSION: Patients with craniomaxillofacial fibrous dysplasia associated with acromegaly may present significant facial deformities that can be satisfactorily treated by cosmetic treatment, especially in patients with psychological problems and severe social acceptance.

Ulnar focal cortical indentation: a progressive, deforming variant of focal fibrocartilaginous dysplasia.

BACKGROUND: Focal fibrocartilaginous dysplasia is a rare growth disturbance of bone resulting in deformity. In the ulna, focal fibrocartilaginous dysplasia is particularly rare, and the characteristic fibroligamentous tether can result in progressive deformity with progressive dislocation of the radial head. The fibroligamentous tether is similar in appearance and function to the Vickers ligament seen in Madelung deformity. The imaging features of ulnar focal fibrocartilaginous dysplasia include a unilateral angular deformity in the ulna with a radiolucent cortical defect and surrounding sclerosis, with secondary radial bowing or radial head dislocation. Focal fibrocartilaginous dysplasia of the ulna has been described using the term ulnar focal cortical indentation. OBJECTIVE: To review the clinical and radiologic features of four cases of ulnar focal cortical indentation occurring in pediatric patients. MATERIALS AND METHODS: We retrospectively reviewed picture archiving and communication system and electronic medical record imaging findings with surgical correlation of ulnar focal cortical indentation at two large specialty pediatric hospitals. RESULTS: Ulnar focal cortical indentation lesions typically arise in the ulnar metaphysis and result in angular forearm deformities with progressive radial deformity including radial head dislocation. Early surgical intervention prevents progression of the deformity and retains range of motion. CONCLUSION: Ulnar focal cortical indentation, although rare, is likely a progressive form of focal fibrocartilaginous dysplasia resulting in significant deformity and disability. Early recognition of the characteristic imaging features is important for early surgical intervention to preserve range of motion and prevent radial head dislocation.

Valgus Osteotomy with Plate-and-Nail Fixation for the Treatment of Proximal Femoral Deformities Due to Fibrous Dysplasia: A Report of Two Cases.

CASE: We describe 2 cases of proximal femoral deformity due to fibrous dysplasia that were corrected with osteotomies and a novel combined construct with a blade plate and a retrograde intramedullary nail. CONCLUSION: A single-stage procedure using a combined construct with a blade plate and a retrograde intramedullary nail is a viable option for correcting and subsequently preventing proximal femoral deformities due to fibrous dysplasia.

Conservative surgical management of simple monostotic fibrous dysplasia of the proximal femur in a 19-year-old basketballer: a case report.

BACKGROUND: Fibrous dysplasia is a rare benign, intramedullary, fibro-osseous lesion. It is thought to be a developmental disorder of bone maturation where normal lamellar bone is replaced by irregular trabecular bone ensnared with fibrous dysplastic tissue that is unable to complete maturation resulting in significant loss of mechanical strength. This, together with the inability to mineralize sufficiently, leads to deformity, pain, and pathological fractures. It typically presents in young adults, with an equal representation in both genders. Surgical intervention is necessary in mild cases with chronic symptoms to prevent pathological fractures or to correct deformities. CASE PRESENTATION: A 19-year-old Chinese woman presented with non-traumatic, nonspecific left hip pain during basketball training. X-rays demonstrated a ground glass lesion, 10 cm in length, in her left femoral neck, which is a classic sign of fibrous dysplasia. No other deformities were noted. She was managed conservatively with analgesia for 6 months; however, her condition did not improve and a decision was made for surgical intervention. The lesion was a type 1 lesion according to the Ippolito radiological classification of fibrous dysplasia, which is a lesion with mild deformities. Therefore, we performed minimal curettage and insertion of a free autologous fibula strut harvested from her left leg, for structural stability. No implants were used. The operation was successful and her postoperative course was uneventful. Histology confirmed the diagnosis of fibrous dysplasia. She achieved partial weight bearing at 4 weeks postoperation, and full weight bearing at 8 weeks, and returned to basketball at 12 weeks. At 1-year follow-up, she returned to competitive basketball and remained pain free with no complications. CONCLUSIONS: Fibrous dysplasia is a rare and benign fibrous tumor of the bone that presents mostly in a young patient population. From our case, we have shown that it is possible to treat young patients with uncomplicated Ippolito type 1 fibrous dysplasia with a minimally invasive approach of using a cortical bone graft for structural augmentation of the affected area, without the use of implants. They are able to fully return to an active and vigorous lifestyle without restriction of activities or long-term risks of orthopedic implant complications.

Fibrous Dysplasia: A Complex Maxillary Reconstruction.

Fibrous dysplasia is a benign tumor of the skeleton. Mostly 2 forms are described: a monostotic and a poliostotic form. The maxilla and the mandible are the most interested of the facial district. The authors report a patient of a huge maxillary fibrous dysplasia. A 63-year-old patient was treated for a 20-year progressive left maxillary neoformation. A total maxillectomy was performed. The defect was reconstructed with a custom-made midface implant associated with a temporal and a pericranial flap.

Functional and radiological outcomes of a minimally invasive surgical approach to monostotic fibrous dysplasia.

BACKGROUND: Reports showing high recurrence rates for intralesional curettage and bone grafting have made the current treatment principle for fibrous dysplasia controversial. This study aimed to report the postoperative clinical outcomes from three minimally invasive surgical strategies we use for monostotic fibrous dysplasia (MFD). PATIENTS AND METHODS: Twelve patients with MFD presenting with no pathologic fracture or deformity and treated with one of three surgical strategies-plain open biopsy, plain alpha-tricalcium phosphate (ATP) reconstruction, and prophylactic bridge plating-were included. There were nine men and three women, with median age of 38 years. Mean follow-up was 88 weeks. Five cases involved the proximal femur, two each involved the femoral and tibial diaphyses, and one each involved the distal humerus, radial diaphysis, and proximal tibia. All cases were reviewed for functional and radiological outcomes. RESULTS: Median time to full activity was 1 day (range 1 to 3) for the plain open biopsy group, while the prophylactic bridge-plating and plain ATP reconstruction groups had longer median recovery times (59 days, range 3 to 143, and 52 days, range 11 to 192, respectively). Musculoskeletal Tumor Society scores at last follow-up were excellent for all the cases (mean 29.6, range 25 to 30). Radiological analysis using Gaski et al.'s criteria showed plain open biopsy resulted in partial resolution of proximal femoral lesions, while ATP reconstruction and prophylactic plating resulted in no change and progression in this lesion site, respectively. For femoral diaphyseal lesions, prophylactic plating resulted in partial resolution, while ATP reconstruction resulted in no change. In the tibial diaphysis, prophylactic plating resulted in partial resolution, while plain open biopsy resulted in no change. For the lesions involving the distal humerus and the proximal tibia, plain open biopsy resulted in partial resolution, while for the radial diaphyseal lesion, ATP reconstruction resulted in no change. Radiological progression was limited in 11 (92%) cases, and none had postoperative complications. CONCLUSION: Plain open biopsies for asymptomatic lesions; prophylactic bridge plating for symptomatic, large diaphyseal lytic lesions; and plain ATP reconstructions for both small and large nondiaphyseal symptomatic lytic lesions may be acceptable alternatives to curettage-incorporating procedures for MFD.

Displasia fibrosa monostótica / Monostotic fibrous dysplasia

No disponible

Fibrous Dysplasia of the Temporal Bone with External Auditory Canal Stenosis and Secondary Cholesteatoma.

Fibrous dysplasia is a slowly progressive benign fibro-osseous disease, rarely occurring in temporal bones. In these cases, most bony lesions developed from the bony part of the external auditory canals, causing otalgia, hearing impairment, otorrhea, and ear hygiene blockade and probably leading to secondary cholesteatoma. We presented the medical history of a 24-year-old woman with temporal monostotic fibrous dysplasia with secondary cholesteatoma. The initial presentation was unilateral conductive hearing loss. A hard external canal tumor contributing to canal stenosis and a near-absent tympanic membrane were found. Canaloplasty and type I tympanoplasty were performed, but the symptoms recurred after 5 years. She received canal wall down tympanomastoidectomy with ossciculoplasty at the second time, and secondary cholesteatoma in the middle ear was diagnosed. Fifteen years later, left otorrhea recurred again and transcanal endoscopic surgery was performed for middle ear clearance. Currently, revision surgeries provide a stable auditory condition, but her monostotic temporal fibrous dysplasia is still in place.

Ureter and Nerve Root Compression Secondary to Expansile Fibrous Dysplasia of the Transverse Process.

Considering their proximity to abdominal viscera, transverse process lesions may pose a diagnostic challenge. We present a case of fibrous dysplasia of the transverse process, causing urinary retention, frequent urinary tract infections, and thigh numbness. This is the first reported case of a transverse process fibrous dysplasia lesion, causing simultaneous urinary retention and neurologic symptoms. Clinicians may consider lesions of the lumbar transverse processes in patients presenting to orthopedic surgeons with urinary symptoms, especially when combined with neurologic symptoms. In these lesions, fibrous dysplasia should be within the differential diagnosis. We discuss the diagnosis and present a brief review of fibrous dysplasia.

Monostotic fibrous dysplasia of the thoracic spine: A case report.

BACKGROUND AND OBJECTIVE: Fibrous dysplasia (FD) is a benign bone lesion manifested by local pain, swelling and deformity change. We report a case of monostotic fibrous dysplasia of the first thoracic vertebrae that treated by radical removal and reconstruction. CASE REPORT: A 29-year-old man with monostotic fibrous dysplasia of the first thoracic vertebrae was admitted to our department because of persistent, dull back pain for 3 months. Radical removal of the first thoracic vertebrae and reconstruction were performed in a combined posterior-anterior approach. This patient experienced complete pain relief without any complication. CONCLUSION: This report presents a rare case of monostotic fibrous dysplasia of the first thoracic vertebrae, with symptoms of chronic back pain that was successfully treated with radical excision and reconstruction, providing a good option to the patient.

Reverse Transfer of the Proximal Vascularized Fibula to Reconstruct the Lateral Malleolus: A Case Report and Literature Review.

Defects of the lateral malleolus and distal fibula occur occasionally, mainly because of severe trauma or wide resection of fibular neoplasms. These bony defects should be reconstructed to avoid persistent pain and to prevent an abnormal gait induced by ankle instability. Various methods of repair have been developed, including allografting, autologous iliac crest transplantation, scapular apophysis transplantation, and arthrodesis and prosthetic reconstruction. A reverse transfer of the proximal vascularized fibula is also effective. Its morphologic advantage is apparent, and the surgery is simple and direct, with no need for vascular anastomosis. We treated recurrent fibrous dysplasia in the distal fibula of a young male with wide resection and reconstruction using reverse transfer of the proximal, vascularized fibula. At the 6-year follow-up examination, the patient had an American Orthopaedic Foot and Ankle Society ankle-hindfoot score of 100, without any apparent complications. Additionally, we critically reviewed other methods for reconstructing the lateral malleolus and distal fibula and have concluded that reverse transfer of the proximal vascularized fibula compares favorably and could be the treatment of choice.