proteus syndrome radiology

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1071, International Journal of Radiology & Radiation Therapy, Vol. Radiologic studies were available for 21 (seven female and 14 male) patients. Photographs of the neck (a) and right foot (b) of an 11-year-old patient show severe deformity of the chest and neck, caused by vertebral anomalies; disproportionate growth of the forefoot (the fifth toe has been amputated because of macrodactyly; see ,,,Fig 4); and plantar cerebriform connective-tissue nevus. Skull abnormalities. Figure 14. Each person with Proteus syndrome is affected in different ways. It is characterized by generalized polyposis of the colon and rectum, macrocephaly, pigmented macules of the penis, lipomas, capillary vascular malformations, and Hashimoto thyroiditis (,22,,23). Skull abnormalities. The first case report (1884) of this disease is credited to Sir Frederick Treves regarding his famous patient, Joseph Merrick (known as the "Elephant Man") 5,6. (d) Axial CT image at the level of the lower thorax depicts hyperexpansion of the left lung and areas of severe scarring and cystic changes in the left lower lobe, as well as mild cystic changes in the right lower lobe. AJR Am J Roentgenol. Hyperostosis. In Proteus syndrome, limb overgrowth is usually absent or mild at birth, whereas in Klippel-Trénaunay syndrome it is present at birth and is commonly severe (, 6). (e) Axial CT image of the abdomen shows increased retroperitoneal fat (arrows); asymmetric development of the paraspinal muscles (arrowheads), with the left side greater than the right; and marked splenomegaly (∗). (c, d) Posteroanterior radiographs of the left hand in another patient at ages 4 years (c) and 16 years (d) show asymmetric and irregular overgrowth of the phalanges, more marked and with accompanying ankylosis of the interphalangeal joints in d.Download as PowerPointOpen in Image It is characterized by … (1)Department of Radiology, Georgetown University Hospital, Washington, DC, USA. Fatty overgrowth. Abnormal vertebral bodies. Sagittal (a) and axial (b) contrast-enhanced T1-weighted MR images of the head in a patient aged 5 years show several focal bone abnormalities, expansile lesions with abnormal accumulation of fatty tissue in several segments of the calvaria, and a focal defect in the outer table of the right occipital bone (arrowhead in b).Download as PowerPointOpen in Image Vascular malformation and hemangiomatosis syndromes: spectrum of imaging manifestations. Axial (a) and sagittal (b) T1-weighted MR images of the thoracic spine in a 6½-year-old female patient show a large lipomatous mass, posterior to the paraspinal muscle fascia, that extends from T6 to L5 (∗), as well as asymmetric fatty infiltration and atrophy of the paraspinal muscles, right more than left (arrowheads in a), and increased fat in the spinal canal (arrow). 12, Revue des Maladies Respiratoires, Vol. Figure 22a. Anteroposterior (a) and left lateral (b) radiographs of the cervical spine in a patient aged 16 years show asymmetric overgrowth of multiple vertebral bodies with resultant dextroscoliosis, hyperlordosis, and abnormal anteroposterior alignment of the upper cervical vertebral bodies, which led to a marked reduction in the patient’s mobility. Progressive skeletal changes. Disorders included in the differential diagnosis of Proteus syndrome are listed in ,Table 3. To help minimize such errors, specific diagnostic criteria have been developed for Proteus syndrome (Appendix 1) … 170, No. Asymmetric overgrowth. Fatty overgrowth. (c) Axial CT image at the level of the upper thorax shows asymmetric overgrowth of fat in the anterior thoracic wall, a focal fatty lesion in the left axilla (arrow), deviation of the mediastinum to the right, and asymmetry of the thorax, with the left hemithorax larger than the right. Posteroanterior radiographs of the left (a) and right (b) hands in a patient aged 2-3 years show asymmetric macrodactyly of the second through fifth left digits and of the second through fourth right digits; clinodactyly of the second, third, and fifth left digits and of the fifth right digit; osteoporosis of the right carpal, metacarpal, and phalangeal bones; and diffuse hypertrophy of the soft tissues in the second right ray.Download as PowerPointOpen in Image Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. Fatty overgrowth. Anteroposterior (a) and left lateral (b) radiographs of the cervical spine in a patient aged 16 years show asymmetric overgrowth of multiple vertebral bodies with resultant dextroscoliosis, hyperlordosis, and abnormal anteroposterior alignment of the upper cervical vertebral bodies, which led to a marked reduction in the patient’s mobility. Cystic and emphysematous lung changes were detected in two of our 21 patients, and pulmonary embolism was the cause of death in two patients. COVID-19 Resources. Progressive skeletal abnormalities such as macrodactyly, scoliosis, asymmetric overgrowth, and limb length discrepancy are the most frequent and striking findings in patients with Proteus syndrome, followed by soft-tissue abnormalities such as fatty, muscular, and vascular malformations. 24 (4): 1051-68. (b) Axial CT image obtained at a level below a, in the neck, shows multiple bilateral asymmetric masses with the attenuation of fat (arrows), more prominent in the left side than the right, and causing deviation of the midline structures in the neck to the right. Viewer. Figure 9a. Figure 13a. (a) Axial T1-weighted MR image obtained in a patient aged 11 years at the level of the mandible shows asymmetric fatty masses (∗) anterior to, and under, the sternocleidomastoid muscles, a finding more prominent in the left side than the right. Differential diagnosis is mostly done on a clinical basis since most of the clinical manifestations are not specific. Other radiographic features include enlarged and dysplastic vertebral bodies, scoliosis, cranial exostosis, osteomas, osteochondromas, enchondromasand genu valgum may also occur. 58, No. Skull abnormalities. (d) Axial CT image at the level of the lower thorax depicts hyperexpansion of the left lung and areas of severe scarring and cystic changes in the left lower lobe, as well as mild cystic changes in the right lower lobe. Case 1: with left facial hemi-hypertrophy, cerebriform connective tissue nevus: virtually pathognomonic, dysregulation of adipose tissue: lipoma, regional absence of fat, overgrowth of fat in the posterior/anterior body wall or un subcutaneous fat of the extremities, vascular malformations: capillary, venous or lymphatic, abnormal facial features, e.g. (a) Axial T1-weighted MR image obtained in a patient aged 11 years at the level of the mandible shows asymmetric fatty masses (∗) anterior to, and under, the sternocleidomastoid muscles, a finding more prominent in the left side than the right. Viewer. (c) Axial CT image at the level of the upper thorax shows asymmetric overgrowth of fat in the anterior thoracic wall, a focal fatty lesion in the left axilla (arrow), deviation of the mediastinum to the right, and asymmetry of the thorax, with the left hemithorax larger than the right. TABLE 3. Proteus syndrome is a sporadic disorder named for its … Abnormal vertebral bodies. This finding, when accompanied by the typical rapid progression seen in Proteus syndrome (,,,Figs 4, ,,,11, ,,,16, ,,,,,18), is a key diagnostic criterion. Check for errors and try again. 4, 13 June 2013 | Skeletal Radiology, Vol. 42, No. Figure 5. Predisposing factors for pulmonary embolism in these patients include vascular malformations, surgical convalescence, and, in severe cases of Proteus syndrome, very restricted mobility (,9). Proteus syndrome is caused by AKT1 gene mutations that occur during early development, the disorder is not inherited and does not run in families. Figure 15a. The study would not have been possible without the support of Proteus syndrome foundations in the United States and the United Kingdom ( www.proteus-syndrome.org and www.proteus-syndrome.org.uk, respectively). Venous malformations. (d) Axial CT image at the level of the lower thorax depicts hyperexpansion of the left lung and areas of severe scarring and cystic changes in the left lower lobe, as well as mild cystic changes in the right lower lobe. 85, No. 19, No. Limb length discrepancy. Viewer. It can affect tissue from any germinal layer. These three investigators, who have extensive experience with Proteus syndrome patients, independently assessed the literature for all articles with the name “Proteus syndrome… Figure 2b. Viewer. Pathological Society of London. T1 - Proteus syndrome T2 - Craniofacial and cerebral MRI AU - Delone, D. R. AU - Brown, W. D. AU - Gentry, L. R. PY - 1999/11/1 Y1 - 1999/11/1 N2 - The Proteus syndrome is a rare hamartoneoplastic syndrome … Although we did not see any of these uncommon tumors in our study population, the radiologist should keep in mind their possible occurrence in association with Proteus syndrome. Limb length discrepancy. … 246, No. This patient attended many different specialists for many years, but unfortunately a certain diagnosis was not revealed. (a) Axial CT image in a patient at age 3 months shows minimal calvarial thickening on the right side. Figure 26e. It is characterized by the progressive and abnormal growth of body tissues, including skin, bones, fatty tissues and blood vessels. Fatty overgrowth. 2. A hallmark of the disorder is the random or mosaic distribution of its manifestations throughout the body. (c) Axial MR image obtained with a short inversion time inversion recovery, or STIR, sequence at a level similar to that in b, shows vascular malformations within the fatty tissue in the retroperitoneum, right psoas muscle, and abdominal wall.Download as PowerPointOpen in Image Axial MR images obtained with a short inversion time inversion recovery sequence in a patient aged 5 years at the levels of the pelvis (a) and thighs (b) show asymmetric fatty infiltration and numerous abnormal vascular structures in the muscles (arrows), more prominent on the left side than on the right. The tissue overgrowth in Klippel-Trénaunay syndrome is usually associated with vascular malformations, whereas in Proteus syndrome the overgrowth of bone and other tissues may occur independently of vascular malformations. 27, No. (a, b) Axial nonenhanced CT images of the thorax (a) and abdomen (b) in a patient aged 14 years show thoracic deformity, including a large asymmetric area with the attenuation of fat along the posterior chest wall and infiltrating the paraspinal muscles bilaterally (arrows), more noticeable in the left side than in the right, which causes elevation of the left scapula away from the posterior chest wall (arrowhead in a); less prominent fatty infiltration along the left lateral chest wall and in the muscles of the anterior chest wall, as well as the posterior, lateral, and anterior abdominal wall and the left anterior rectus abdominis muscle; and enlargement of the right psoas muscle with fatty infiltration that surrounds multiple serpentine blood vessels. 5, 1 March 2008 | Radiology, Vol. Note also the rib asymmetry and thoracic scoliosis in a.Download as PowerPointOpen in Image MR imaging was useful also for identifying abnormalities in the underlying bones of the foot (,Fig 10). (b) Axial CT image obtained at a level below a, in the neck, shows multiple bilateral asymmetric masses with the attenuation of fat (arrows), more prominent in the left side than the right, and causing deviation of the midline structures in the neck to the right. Axial (a) and sagittal (b) T1-weighted MR images of the thoracic spine in a 6½-year-old female patient show a large lipomatous mass, posterior to the paraspinal muscle fascia, that extends from T6 to L5 (∗), as well as asymmetric fatty infiltration and atrophy of the paraspinal muscles, right more than left (arrowheads in a), and increased fat in the spinal canal (arrow). Diffuse and infiltrating vascular malformations (lymphatic, venous, or capillary) were present in seven (33%) of our 21 patients. (b) Right lateral radiograph obtained at age 8½ years shows progressive enlargement of the vertebral bodies with resultant fixed hyperextension of the upper cervical spine and hyperflexion of the lower cervical spine, which led to a reduction in the patient’s mobility.Download as PowerPointOpen in Image Abnormal vertebral bodies. (b) Right lateral radiograph obtained at age 8½ years shows progressive enlargement of the vertebral bodies with resultant fixed hyperextension of the upper cervical spine and hyperflexion of the lower cervical spine, which led to a reduction in the patient’s mobility. (e) Axial CT image of the abdomen shows increased retroperitoneal fat (arrows); asymmetric development of the paraspinal muscles (arrowheads), with the left side greater than the right; and marked splenomegaly (∗). Figure 26e. (a) Axial CT image in a patient at age 3 months shows minimal calvarial thickening on the right side. (a, b) Axial nonenhanced CT images of the thorax (a) and abdomen (b) in a patient aged 14 years show thoracic deformity, including a large asymmetric area with the attenuation of fat along the posterior chest wall and infiltrating the paraspinal muscles bilaterally (arrows), more noticeable in the left side than in the right, which causes elevation of the left scapula away from the posterior chest wall (arrowhead in a); less prominent fatty infiltration along the left lateral chest wall and in the muscles of the anterior chest wall, as well as the posterior, lateral, and anterior abdominal wall and the left anterior rectus abdominis muscle; and enlargement of the right psoas muscle with fatty infiltration that surrounds multiple serpentine blood vessels. Radiographics. Figure 17. Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. Axial CT. 2, Journal of Human Genetics, Vol. In Parkes Weber syndrome (a variant of Klippel-Trénaunay syndrome), limb hypertrophy is associated with arteriovenous malformations (,19). Bone malformations in Proteus syndrome: an analysis of bone structural changes and their evolution during growth. Anteroposterior radiographs of the pelvis and thighs (a) and the legs and feet (b) of a 12-year-old male patient show asymmetric overgrowth of bones and soft tissues in the right side of the pelvis and the right lower extremity, limb length discrepancy, and bowing in the left femur and right fibula. (e) Axial CT image of the abdomen shows increased retroperitoneal fat (arrows); asymmetric development of the paraspinal muscles (arrowheads), with the left side greater than the right; and marked splenomegaly (∗). AU - Delone, D. R. AU - Brown, W. D. AU - Gentry, L. R. PY - 1999/11/1. 4, Journal of the Japanese Society of Intensive Care Medicine, Vol. Skull abnormalities. (e) Axial CT image of the abdomen shows increased retroperitoneal fat (arrows); asymmetric development of the paraspinal muscles (arrowheads), with the left side greater than the right; and marked splenomegaly (∗). We saw muscular calcifications in three (14%) of our 21 patients. Cystic lung changes and pulmonary emboli (,,,,,,,Fig 26) also have been reported to occur in association with Proteus syndrome (,5,,7,–,9). Anticoagulant therapy was begun immediately after diagnosis of embolism, and both patients recovered. Figure 12a. Figure 26f. The visceral anomalies most frequently seen in our patients (,,,,,,,Figs 26, ,27) were splenomegaly, seen in five (29%) of 17 patients with visceral anomalies; and asymmetric megalencephaly, white-matter abnormalities, and nephromegaly, each of which was seen in four (24%) of these 17 patients. (e) Axial CT image of the abdomen shows increased retroperitoneal fat (arrows); asymmetric development of the paraspinal muscles (arrowheads), with the left side greater than the right; and marked splenomegaly (∗). (c) Axial CT image at the level of the upper thorax shows asymmetric overgrowth of fat in the anterior thoracic wall, a focal fatty lesion in the left axilla (arrow), deviation of the mediastinum to the right, and asymmetry of the thorax, with the left hemithorax larger than the right. (b) Axial MR image obtained with a fluid-attenuated inversion recovery, or FLAIR, sequence in the same patient at age 8 years shows expansile calvarial lesions with the signal intensity of fat in the frontal and right parietal bones (arrows); a lesion in the right parieto-occipital junction, probably a cavernous vascular malformation (arrowhead); and bilateral abnormalities in periventricular and deep white-matter signal intensities. [ 2004 ]. Deformities observed in the nasal bridge, alveolar dental ridges, and, rarely, external auditory canal were related to overgrowth of bone. 3, Dentomaxillofacial Radiology, Vol. Asymmetric overgrowth. Figure 21a. Viewer. 13, Blood Coagulation & Fibrinolysis, Vol. (a, b) Axial nonenhanced CT images of the thorax (a) and abdomen (b) in a patient aged 14 years show thoracic deformity, including a large asymmetric area with the attenuation of fat along the posterior chest wall and infiltrating the paraspinal muscles bilaterally (arrows), more noticeable in the left side than in the right, which causes elevation of the left scapula away from the posterior chest wall (arrowhead in a); less prominent fatty infiltration along the left lateral chest wall and in the muscles of the anterior chest wall, as well as the posterior, lateral, and anterior abdominal wall and the left anterior rectus abdominis muscle; and enlargement of the right psoas muscle with fatty infiltration that surrounds multiple serpentine blood vessels. Figure 26c. Figure 7. (a) Axial T1-weighted MR image obtained in a patient aged 11 years at the level of the mandible shows asymmetric fatty masses (∗) anterior to, and under, the sternocleidomastoid muscles, a finding more prominent in the left side than the right. Imaging manifestations in Proteus syndrome: an unusual multisystem developmental disorder. However, the differential diagnosis includes: The name of this syndrome comes from the Greek god of the sea Proteus who had the ability to change his shape to avoid capture, it was proposed by Wiedemann et al. Macrodactyly and clinodactyly. Affected persons have some cells with a normal copy of this regulatory gene and some cells with the abnormal gene (mosaic). 4, 15 March 2011 | American Journal of Medical Genetics Part A, Vol. Figure 27. Congenital syndrome described by Cohen et al., 1 in 1979, being called Proteus (also known as Wiedmann Syndrome) in 1983 by Wiedmann 2 because of the multiple variations with which it may present itself (Proteus … Axial MR images obtained with a short inversion time inversion recovery sequence in a patient aged 5 years at the levels of the pelvis (a) and thighs (b) show asymmetric fatty infiltration and numerous abnormal vascular structures in the muscles (arrows), more prominent on the left side than on the right. Figure 1a. One of the most characteristic findings in Proteus syndrome is the disorganization and distortion of skeletal features (,Figs 6, ,10, ,17), a finding that contrasts strikingly with more common forms of osseous overgrowth in which the enlarged bones retain their normal proportional relationships. T2 - Craniofacial and cerebral MRI. Hyperostosis. 70, No. Figure 7a. The unusual and highly variable manifestations of the syndrome frequently lead to misdiagnosis: Patients may have other disorders that are incorrectly diagnosed as Proteus syndrome, or may have Proteus syndrome that is incorrectly diagnosed as another disease. Overgrowth of fat and muscle may coexist and result in abnormally large muscle groups   that contain interspersed fat (,,,Figs 21,,,–,,,,23). Some people may have overgrown limbs while others experience an overgrowth of the skull. Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. Although the manifestations of Proteus syndrome are highly variable, accurate diagnosis is possible if standard diagnostic criteria are followed and if disease features are assessed in comparison with those found in similar syndromes. Skull abnormalities. Progressive skeletal changes. Anteroposterior radiograph of the knees in a patient aged 20 years shows asymmetric overgrowth of the soft tissues in the left lower extremity, as well as hyperostosis, deformity, and osteoporosis of the left distal femur and proximal tibia.Download as PowerPointOpen in Image Proteus syndrome is a congenital disorder of unknown etiology, and it is the prototype of overgrowth syndromes. Commonly referred to as moccasin sole or cerebriform nevus, such findings were seen in nine (43%) of our patients. Neck deformity and connective-tissue nevus. When all cells have the abnormal gene, the condition is not compatible with life. (a) Axial T1-weighted MR image obtained in a patient aged 11 years at the level of the mandible shows asymmetric fatty masses (∗) anterior to, and under, the sternocleidomastoid muscles, a finding more prominent in the left side than the right. Vertebral anomalies and connective-tissue nevi are characteristic features of Proteus syndrome.Download as PowerPointOpen in Image in 1983. 3, 15 December 2017 | The Cleft Palate-Craniofacial Journal, Vol. Macrodistrofia lipomatosa del pie asociada a lipomatosis del nervio plantar, Vascular Malformation and Hemangiomatosis Syndromes: Spectrum of Imaging Manifestations, A Case of Proteus Syndrome with Lateral Embryonal Vein and Frontal Intraosseous Lipoma, Dentomaxillofacial imaging in Proteus syndrome, Imagerie des anomalies vasculaires des tissus mous : diagnostic et traitement. 1067, 23 August 2016 | American Journal of Medical Genetics Part A, Vol. Anteroposterior radiograph of the knees in a patient aged 20 years shows asymmetric overgrowth of the soft tissues in the left lower extremity, as well as hyperostosis, deformity, and osteoporosis of the left distal femur and proximal tibia. The name thus refers to the unpredictable asymmetric gigantism/hemihypertrophy associated with this disease. The specific genetic characteristics of Bannayan-Riley-Ruvalcaba syndrome and its distinct phenotype set it apart from Proteus syndrome. Pediatric radiology 2007; 37:829–835. 8. The extremely rare Proteus Syndrome is a hamartomatous congenital syndrome with substantial vari- ability between clinical patient presentations. 3, 7 January 2012 | Japanese Journal of Radiology, Vol. Figure 25. Viewer. (c, d) Posteroanterior radiographs of the left hand in another patient at ages 4 years (c) and 16 years (d) show asymmetric and irregular overgrowth of the phalanges, more marked and with accompanying ankylosis of the interphalangeal joints in d. Figure 19. 33, No. Viewer. 5, American Journal of Medical Genetics Part A, Vol. (b) Anteroposterior radiograph obtained 14 months later shows progressive overgrowth in the right foot; redundant lobulated plantar skin, characteristic of plantar cerebriform connective-tissue nevus (arrowheads); and macrodactyly of the right fifth toe, with a notched deformity in the midportion of the proximal phalanx (arrow). ), TABLE 2. (b) Axial MR image obtained with a fluid-attenuated inversion recovery, or FLAIR, sequence in the same patient at age 8 years shows expansile calvarial lesions with the signal intensity of fat in the frontal and right parietal bones (arrows); a lesion in the right parieto-occipital junction, probably a cavernous vascular malformation (arrowhead); and bilateral abnormalities in periventricular and deep white-matter signal intensities.Download as PowerPointOpen in Image 55, No. Radiologic manifestations of Proteus syndrome. Posteroanterior radiographs of the left (a) and right (b) hands in a patient aged 2-3 years show asymmetric macrodactyly of the second through fifth left digits and of the second through fourth right digits; clinodactyly of the second, third, and fifth left digits and of the fifth right digit; osteoporosis of the right carpal, metacarpal, and phalangeal bones; and diffuse hypertrophy of the soft tissues in the second right ray. Abnormal vertebral bodies. Sagittal (a) and axial (b) contrast-enhanced T1-weighted MR images of the head in a patient aged 5 years show several focal bone abnormalities, expansile lesions with abnormal accumulation of fatty tissue in several segments of the calvaria, and a focal defect in the outer table of the right occipital bone (arrowhead in b).Download as PowerPointOpen in Image Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. Anteroposterior (a) and left lateral (b) radiographs of the lumbar spine in a patient aged 6 years show asymmetric overgrowth of multiple vertebral bodies and increased vertebral height, particularly of L3 and L4; lumbarization of S1; and posterior scalloping of all of the lumbar vertebral bodies, as well as S1. Asymmetric overgrowth. In this respect, our findings did not bear out previously published reports of patients with Proteus syndrome and spinal compression from invasive lipomas (,15,,17). (c) Axial CT image at the level of the upper thorax shows asymmetric overgrowth of fat in the anterior thoracic wall, a focal fatty lesion in the left axilla (arrow), deviation of the mediastinum to the right, and asymmetry of the thorax, with the left hemithorax larger than the right. Viewer. Progressive irregular and dysplastic overgrowth of bone, which is typical of Proteus syndrome (,,,Figs 4, ,,,11, ,,,16, ,,,,,18), is not seen in Klippel-Trénaunay syndrome and therefore is a key radiologic distinction. 4, Current Cardiovascular Imaging Reports, Vol. Viewer. The extremely rare Proteus Syndrome is a hamartomatous congenital syndrome with substantial variability between clinical patient presentations. The disorders most commonly confused with Proteus syndrome are Klippel-Trénaunay syndrome, neurofibromatosis type 1, and hemihyperplasia–multiple lipomatosis syndrome (,6,,12). Blood vessels and internal organs can also be affected. It is suspected to be a genetic condition, but a particular gene is not currently identified. 6. Figure 26c. Diagnostic Criteria for Proteus Syndrome, Note.—Diagnosis is made in the presence of the general (mandatory) criteria—mosaic distribution of lesions and progressive course and sporadic occurrence—plus either the single sign from category A, two signs from category B, or three signs from category C. (Adapted, with permission, from reference 6. None of our patients, however, had symptoms of spinal compromise. Note the asymmetry of the ossification centers in the second and third rays (arrows).Download as PowerPointOpen in Image Viewer. Organs and tissues affected by the disease grow out of proportion to the rest of the body. Note also the rib asymmetry and thoracic scoliosis in a.Download as PowerPointOpen in Image Viewer. Anteroposterior (a) and left lateral (b) radiographs of the cervical spine in a patient aged 16 years show asymmetric overgrowth of multiple vertebral bodies with resultant dextroscoliosis, hyperlordosis, and abnormal anteroposterior alignment of the upper cervical vertebral bodies, which led to a marked reduction in the patient’s mobility. A-C. Hyperostosis. (b) Axial MR image obtained with a fluid-attenuated inversion recovery, or FLAIR, sequence in the same patient at age 8 years shows expansile calvarial lesions with the signal intensity of fat in the frontal and right parietal bones (arrows); a lesion in the right parieto-occipital junction, probably a cavernous vascular malformation (arrowhead); and bilateral abnormalities in periventricular and deep white-matter signal intensities. (d) Axial CT image at the level of the lower thorax depicts hyperexpansion of the left lung and areas of severe scarring and cystic changes in the left lower lobe, as well as mild cystic changes in the right lower lobe. (a) Right lateral radiograph obtained in a patient at age 6 years shows enlargement but relatively normal alignment of the cervical vertebral bodies. Cases of PS from 2005 … Appropriate techniques for managing these lesions cannot be devised without additional data. (e) Axial CT image of the abdomen shows increased retroperitoneal fat (arrows); asymmetric development of the paraspinal muscles (arrowheads), with the left side greater than the right; and marked splenomegaly (∗).

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