Viewer. Figure 18d. 27, No. The diagnostic tests recommended for use in patient evaluation include skeletal surveys to characterize skeletal manifestations, magnetic resonance (MR) imaging of the abdomen and pelvis to exclude asymptomatic but potentially aggressive intraabdominal lipomas, MR imaging of the central nervous system to help identify abnormalities associated with neurologic symptoms, and high-spatial-resolution computed tomography (CT) of the chest to further characterize abnormal findings at prior chest radiography or pulmonary function testing. (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. Abnormal vertebral bodies. (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. 2, Journal of Pediatric Orthopaedics, Vol. The disease commonly progresses rapidly in childhood but may slow or stabilize during early adolescence. (a, b) Anteroposterior radiographs of the left foot in a patient at ages 2 years (a) and 5 years (b) show progressive and disproportionate growth of the metatarsal and phalangeal bones of the toes, macrodactyly and clinodactyly, and a cerebriform connective-tissue nevus (arrowheads in b). A-C. 10, 18 December 2015 | The Indian Journal of Pediatrics, Vol. 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. Such a mutation could affect local production or regulation of tissue growth factor receptors (,1,,12). These changes were readily detected both with MR imaging and with CT and were obvious at clinical evaluation. … Because the manifestations of Proteus syndrome are highly variable and many are found also in other overgrowth syndromes, diagnosis can be difficult. (1)Department of Radiology, Georgetown University Hospital, Washington, DC, USA. We present a case with severe, characteristic findings. 4, Journal of the Japanese Society of Intensive Care Medicine, Vol. 33, No. Acute pulmonary embolism was diagnosed also in two other patients in this cohort during the study period: One of the two, a 16-year-old male patient, experienced two episodes of pulmonary embolism while recovering from endoscopy for gastrointestinal hemorrhage. Figure 11b. TABLE 3. (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 6. Figure 9b. Multiple calcified phleboliths within pelvic venous malformations can be seen on radiographs of the abdomen (,19). (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. The extremely rare Proteus Syndrome is a hamartomatous congenital syndrome with substantial variability between clinical patient presentations. (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. Figure 15a. 1017, 13 March 2012 | Fetal and Pediatric Pathology, Vol. 2014 | Volume 9 | Issue 2 Figure 6. Figure 10. Progressive skeletal changes. Skull abnormalities. 55, No. Viewer. (a) Anteroposterior radiograph of the right foot (same patient as in ,,,Fig 1), obtained at age 5 years, shows minimal overgrowth of bone. Viewer. 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.Download as PowerPointOpen in Image 9, Journal of Thoracic Imaging, Vol. Proteus syndrome is a very rare condition with a prevalence less than 1/1,000,000. Viewer. Figure 8. (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. 1 This syndrome was first delineated by Cohen and Hayden 2 in 1979, who reported a newly recognized disorder in two patients. (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. Proteus syndrome: a newly recog- nized hamartomatous syndrome with significant craniofacial dysmorphology. Proteus syndrome is characterized by excessive growth of a part or portion of the body. Rapid progression of cervical spine overgrowth. (f) Contrast-enhanced CT image of the chest obtained 4 years later shows filling defects in the lower lobes of the right and left lungs because of emboli in the pulmonary arteries, as well as a small left pleural effusion.Download as PowerPointOpen in Image 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. 5, 1 March 2008 | Radiology, Vol. 5, The British Journal of Radiology, Vol. 143A, No. 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 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.Download as PowerPointOpen in Image Fatty infiltration of muscles, and vascular malformations. 3, Dentomaxillofacial Radiology, Vol. Sagittal T1-weighted MR image of the right foot in a patient aged 7 years shows disproportionate and dysmorphic enlargement of the calcaneus (∗) and an irregular overgrowth of the plantar soft tissues that represents cerebriform connective-tissue nevus (arrowheads). 2. (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). Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. 1, The British Journal of Radiology, Vol. These overgrowths usually … Viewer. The study was carried out by Turner et al. It is suspected to be a genetic condition, but a particular gene is not currently identified. Abnormal vertebral bodies. Figure 7a. 3, American Journal of Roentgenology, Vol. (a, b) Anteroposterior radiographs of the left foot in a patient at ages 2 years (a) and 5 years (b) show progressive and disproportionate growth of the metatarsal and phalangeal bones of the toes, macrodactyly and clinodactyly, and a cerebriform connective-tissue nevus (arrowheads in b). Figure 23b. This patient attended many different specialists for many years, but unfortunately a certain diagnosis was not revealed. 4, 15 March 2011 | American Journal of Medical Genetics Part A, Vol. Skull abnormalities. Axial T1-weighted MR image through the level of the pelvis in a patient aged 8 years shows asymmetric adipose overgrowth along the anterior and lateral portion of the abdominal wall (∗), asymmetry of the abdominal wall musculature (arrowheads), and fatty infiltration of the paraspinal muscles (arrows).Download as PowerPointOpen in Image (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 (∗). Viewer. Viewer. Viewer. Abnormal vertebral bodies. (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 Viewer. 3, 7 January 2012 | Japanese Journal of Radiology, Vol. 3, 15 December 2017 | The Cleft Palate-Craniofacial Journal, Vol. Hyperostosis. (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. 31, No. Jamis-dow CA, Turner J, Biesecker LG et-al. 1, American Journal of Medical Genetics, Vol. Viewer. Thieme Publishing Group. Figure 21a. 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. (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. 83, No. (a) Right lateral radiograph obtained in a patient at age 6 years shows enlargement but relatively normal alignment of the cervical vertebral bodies. Spinal deformities also are a frequent finding in Proteus syndrome, and progressive spinal deformity is usually noted in childhood (,,,Fig 11). 7, 23 October 2013 | Clinical Genetics, Vol. In an analysis of 205 cases of “Proteus syndrome” from the literature, only 47.3% of the cases actually had bona fide Proteus syndrome. Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. Reports of Societies. Viewer. Viewer. 85, No. Progressive skeletal changes. The extremely rare Proteus Syndrome is a hamartomatous congenital syndrome with substantial vari- ability between clinical patient presentations. Skull abnormalities. Viewer. 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. (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 (∗). Axial MR image obtained with a short inversion time inversion recovery sequence at the level of the thighs in a patient aged 27 years shows multiple enlarged veins in the subcutaneous tissues and posterior muscles of the right thigh (arrows), as well as a slight enlargement of the right thigh with increased subcutaneous fat, which causes a mild asymmetry in the cross-sectional area of the thighs.Download as PowerPointOpen in Image Axial T1-weighted MR image of the hands of a 28-year-old female patient shows marked asymmetry due to increased fatty components in the soft tissues of the right hand. Viewer. Figure 23c. The most common causes of premature death in Proteus syndrome are pulmonary embolism and respiratory failure (,9). 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. Proteus syndrome is a complex disorder consisting variably of disproportionate, asymmetric overgrowth of body parts, particularly involving the skeleton; cer- 1113, Topics in Magnetic Resonance Imaging, Vol. It is a complex disorder with multisystem involvement and great clinical variability. 2. 34, No. (a, b) Anteroposterior radiographs of the left foot in a patient at ages 2 years (a) and 5 years (b) show progressive and disproportionate growth of the metatarsal and phalangeal bones of the toes, macrodactyly and clinodactyly, and a cerebriform connective-tissue nevus (arrowheads in b). 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).Download as PowerPointOpen in Image Adipose tissue overgrowth is most commonly manifested as asymmetry in the anterior or posterior body wall or in the subcutaneous fat of the extremities (,Figs 19,–,,,21). Some people may have overgrown limbs while others experience an overgrowth of the skull. 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. Figure 13a. Limb length discrepancy. Figure 15b. 170, No. Knowledge of the multiple highly specific radiologic manifestations of Proteus syndrome is therefore essential for diagnosis of this condition. Proteus syndrome is a rare disorder with a genetic background that can cause tissue overgrowth involving all three embryonic lineages. Eight of the 14 male patients underwent scrotal ultrasonography. The features of Proteus syndrome indicate that the condition may be caused by a somatic alteration in a gene, but no specific genetic mutation has yet been identified. Figure 11a. (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 18c. Viewer. (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. 1885;36:494-8. 4, Dentomaxillofacial Radiology, Vol. 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. It is characterized by … Vascular malformation and hemangiomatosis syndromes: spectrum of imaging manifestations. Some other patients with PTEN mutations have atypical (non–Bannayan-Riley-Ruvalcaba syndrome) overgrowth phenotypes. Fatty infiltration of muscles and vascular malformations. Neck deformity and connective-tissue nevus. Foi descrita pela … Radiologic studies were available for 21 (seven female and 14 male) patients. Figure 17. Fatty infiltration of muscles and vascular malformations. 94, No. According to Savannahian Daniel DeLoach, 27, “there are always people out there in much worse situations.” Here’s his: 105 surgeries — so far — including two spinal cord surgeries, three body casts, two near-death close calls, one kidney, hindered lungs and breathing, swollen feet AU - Delone, D. R. AU - Brown, W. D. AU - Gentry, L. R. PY - 1999/11/1. Figure 11a. 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.Download as PowerPointOpen in Image Viewer. 2014, Journal of the Korean Society of Radiology, Vol. The highly variable clinical manifestations frequently lead to initial misdiagnosis 3. (a) Axial CT image in a patient at age 3 months shows minimal calvarial thickening on the right side. Employee/Occupational Health: 684-8115. 137C, No. 4, 1 August 2010 | The Neuroradiology Journal, Vol. MR imaging was useful also for identifying abnormalities in the underlying bones of the foot (,Fig 10). Some affected bones have a thin cortex and coarse trabeculation (,Fig 17), and some appear demineralized (,,,Figs 2, ,6), findings that may be attributable to disuse. 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. Figure 23a. 85, No. Note also the rib asymmetry and thoracic scoliosis in a.Download as PowerPointOpen in Image (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. Rapid progression of macrodactyly and cerebriform connective-tissue nevus. (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).Download as PowerPointOpen in Image Macrodactyly (,,,Figs 2,–,,,4) is one of the most striking manifestations of Proteus syndrome. The adipose overgrowth in this patient extended from the hand into the forearm.Download as PowerPointOpen in Image Viewer, Radiological Aspect of Klippel-Trénaunay Syndrome: A Case Series With Review of Literature, RASA1-Related Parkes Weber Syndrome in a Neonate, Protean manifestations of Proteus syndrome, Hamartomas from head to toe: an imaging overview, Musculoskeletal and overgrowth syndromes associated with cutaneous abnormalities, Somatic However, characteristically, Proteus syndrome is considered when there is hemimegalencephaly, extensive lymphangitic or vascular malformations and asymmetric hemihypertrophy 1. (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. (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. Proteus syndrome (PS) is a rare, progressive disorder that manifests as asymmetric, disproportionate overgrowth affecting tissues derived from any germline layer. (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 The AKT1 gene is … Figure 4a. It causes an overgrowth of skin, bones, blood vessels, and fatty and connective tissue. (a) Anteroposterior radiograph of the right foot (same patient as in ,,,Fig 1), obtained at age 5 years, shows minimal overgrowth of bone. Overgrowth of fat and muscle may coexist and result in abnormally large muscle groups   that contain interspersed fat (,,,Figs 21,,,–,,,,23). Hyperostosis. Figure 3. Figure 9b. (a, b) Anteroposterior radiographs of the left foot in a patient at ages 2 years (a) and 5 years (b) show progressive and disproportionate growth of the metatarsal and phalangeal bones of the toes, macrodactyly and clinodactyly, and a cerebriform connective-tissue nevus (arrowheads in b). (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. To help minimize such errors, participants in a workshop about Proteus syndrome held at the National Institutes of Health in 1998 (,6) developed lists of diagnostic criteria (,Table 1) and of diseases that should be considered in the differential diagnosis (,Table 2), as well as guidelines for evaluation of patients (,Table 3). (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 (∗). Rapid progression of cervical spine overgrowth. When the infant begins to grow, the condition will become apparent — typically between 6 and 18 … The disease causes tissue overgrowth in a mosaic pattern and may affect tissues derived from any germinal layer. Figure 17. Progressive skeletal changes. 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. 30, No. Coronal and axial T1-weighted MR images of the pelvis and thighs (a) and the middle thighs (b) in a patient aged 11 years show muscle and fat overgrowth in the left thigh and mild fatty infiltration (arrow in b) in the muscles of the left thigh. Several articles about the differential diagnosis of Proteus syndrome have been published (,6,,12). Cystic and emphysematous lung changes were detected in two of our 21 patients, and pulmonary embolism was the cause of death in two patients. (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.Download as PowerPointOpen in Image Bone malformations in Proteus syndrome: an analysis of bone structural changes and their evolution during growth. dolichocephaly, low nasal bridge, anteverted nares. Kormano M, Lindgren I. Radiological Findings in Skin Diseases and Related Conditions. Radiographic features are not specific and refer to any clinical characteristics. (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 18b. Rare overgrowth syndrome thought to affect fewer than 500 individuals in the second and third rays arrows. And venous malformations with phleboliths in the differential diagnosis of embolism, and pulmonary.... Ct Image in a patient at age 3 months shows minimal calvarial thickening on the right anterior chest! Patient at age 3 months shows minimal calvarial thickening on the right and left of... This entity into the forearm.Download as PowerPointOpen in Image Viewer was useful also for identifying abnormalities childhood! 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proteus syndrome radiology

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