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Chest: Neurofibromatosis Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Chest ❯ Neurofibromatosis

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  • The most frequent findings were the following: ribs erosion (19.8%), pectus excavatum (12.0%), kyphoscoliosis (3.5%) and posterior mediastinal masses (7.1%). Such results suggest that posterior mediastinal masses (neurofibroma and meningocele) should be included as a diagnostic criterion of type 1 neurofibromatosis, in conjunction with dysplasia of the sphenoid wing, pseudoarthrosis and thinning of long bone cortex, as defined by the National Institutes of Health.

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • Neurofibromatosis (NF) is an autosomal dominant disease that was first described by Friedrich von Recklinghausen in 1882. Several types of NF have been described, and type 1 (NF1), also denominated peripheral or classic NF, is the most common, occurring in 1:3,000 individuals. One of the main characteristics of this disease is systemic and progressive involvement, with impaired neurological functions and physical deformity.

    The diagnostic criteria for NF1 were defined by the National Institutes of Health (NIH) in 1987 and updated three years later(5). Main clinical presentations of NF1 include café-au-lait spots, cutaneous neurofibromas, ephelides or freckling in axillary and/or inguinal regions, plexiform neurofibromas and Lisch nodules

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • “The commonest alterations include scoliosis, kyphoscoliosis, erosion of the posterior wall of vertebral bodies, erosion of the anterior wall of vertebral bodies, widening of intervertebral foramina, growth disorders, pseudoarthrosis, thinning of long bone cortices, bone cystic lesions, costal arch erosion, thinning of pedicles, sphenoid wing dysplasia, cranial osteolytic lesions, facial/mandibular deformities, subperiosteal bone proliferation, bone compression due to soft-tissue tumors (neurofibromas, dura mater dysplasias, intrathoracic meningocele)”

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • “Among the alterations found in the analysis of chest radiographs, costal arch irregularities were the most common ones (19.1%). This abnormality may occur due to a primary dysplastic defect in bone formation or by erosion of an intercostal neurofibroma(8,10,14). A "twisted-ribbon" appearance of the ribs is frequently reported”

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • “About 80% of patients presenting with intrathoracic meningoceles have NF1(25,26). Two or more skin neurofibromas or one plexiform neurofibroma are diagnostic criteria of NF1 as defined by the NIH). Thus, the presence of neurofibromas in the paravertebral regions should be considered as of equal value in the diagnosis of NF1. No information on the incidence of posterior mediastinal masses in the general population was found in the literature.” 

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • “Our study indicates an incidence of PE in inpatients of 19.2 %, in ED patients of 6.7 %, and outpatients of 6.4 %. This compares to Mamlouk where the incidences were 13.46 % in inpatients and 6.36 % in ED patients. The large adult sample size of our study resulted in high confidence levels. ” 

    Incidence of pulmonary emboli on chest computed tomography angiography based upon referral patterns Meesa IR et al. Emerg Radiol (2016) 23::251–254
  • The most frequent findings were the following: ribs erosion (19.8%), pectus excavatum (12.0%), kyphoscoliosis (3.5%) and posterior mediastinal masses (7.1%). Such results suggest that posterior mediastinal masses (neurofibroma and meningocele) should be included as a diagnostic criterion of type 1 neurofibromatosis, in conjunction with dysplasia of the sphenoid wing, pseudoarthrosis and thinning of long bone cortex, as defined by the National Institutes of Health.

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • Neurofibromatosis (NF) is an autosomal dominant disease that was first described by Friedrich von Recklinghausen in 1882. Several types of NF have been described, and type 1 (NF1), also denominated peripheral or classic NF, is the most common, occurring in 1:3,000 individuals. One of the main characteristics of this disease is systemic and progressive involvement, with impaired neurological functions and physical deformity.

    The diagnostic criteria for NF1 were defined by the National Institutes of Health (NIH) in 1987 and updated three years later(5). Main clinical presentations of NF1 include café-au-lait spots, cutaneous neurofibromas, ephelides or freckling in axillary and/or inguinal regions, plexiform neurofibromas and Lisch nodules

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • “The commonest alterations include scoliosis, kyphoscoliosis, erosion of the posterior wall of vertebral bodies, erosion of the anterior wall of vertebral bodies, widening of intervertebral foramina, growth disorders, pseudoarthrosis, thinning of long bone cortices, bone cystic lesions, costal arch erosion, thinning of pedicles, sphenoid wing dysplasia, cranial osteolytic lesions, facial/mandibular deformities, subperiosteal bone proliferation, bone compression due to soft-tissue tumors (neurofibromas, dura mater dysplasias, intrathoracic meningocele)”

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • “Among the alterations found in the analysis of chest radiographs, costal arch irregularities were the most common ones (19.1%). This abnormality may occur due to a primary dysplastic defect in bone formation or by erosion of an intercostal neurofibroma(8,10,14). A "twisted-ribbon" appearance of the ribs is frequently reported”

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010
  • “About 80% of patients presenting with intrathoracic meningoceles have NF1(25,26). Two or more skin neurofibromas or one plexiform neurofibroma are diagnostic criteria of NF1 as defined by the NIH). Thus, the presence of neurofibromas in the paravertebral regions should be considered as of equal value in the diagnosis of NF1. No information on the incidence of posterior mediastinal masses in the general population was found in the literature.”

    Type 1 neurofibromatosis: radiological findings of the chest* Marcos Pontes Muniz et al. Radiol Bras vol.43 no.3 São Paulo May/June 2010

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