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Radiologic Manifestations of Proteus Syndrome¹

¹ From the Department of Radiology, Georgetown University Hospital, Washington, DC (C.A.J.D.); Genetic Diseases Research Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Md (J.T., L.G.B.); and Department of Radiology, Clinical Center, National Institutes of Health, Bldg 10, Room 1C-660, MSC 1182, Bethesda, MD 20892 (P.L.C.). Received October 7, 2003; revision requested November 17; revision received March 2, 2004 and accepted March 9. Address correspondence to P.L.C. (e-mail: pchoyke@cc.nih.gov).

View larger version (122K): [in a new window]   Figure 1a.  Neck deformity and connective-tissue nevus. 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. Vertebral anomalies and connective-tissue nevi are characteristic features of Proteus syndrome.

View larger version (86K): [in a new window]   Figure 1b.  Neck deformity and connective-tissue nevus. 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. Vertebral anomalies and connective-tissue nevi are characteristic features of Proteus syndrome.

View larger version (93K): [in a new window]   Figure 2a.  Macrodactyly and clinodactyly. 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.

View larger version (92K): [in a new window]   Figure 2b.  Macrodactyly and clinodactyly. 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.

View larger version (124K): [in a new window]   Figure 3.  Asymmetric overgrowth and macrodactyly. Posteroanterior radiograph of the hands in a male patient aged 2-3 years shows asymmetric overgrowth of soft tissues and metacarpal and phalangeal bones in the left hand, particularly prominent in the second and third rays, and less-prominent macrodactyly in the fourth and fifth rays in the right hand. Note the asymmetry of the ossification centers in the second and third rays (arrows).

View larger version (88K): [in a new window]   Figure 4a.  Rapid progression of macrodactyly and cerebriform connective-tissue nevus. (a) Anteroposterior radiograph of the right foot (same patient as in Fig 1), obtained at age 5 years, shows minimal overgrowth of bone. (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).

View larger version (76K): [in a new window]   Figure 4b.  Rapid progression of macrodactyly and cerebriform connective-tissue nevus. (a) Anteroposterior radiograph of the right foot (same patient as in Fig 1), obtained at age 5 years, shows minimal overgrowth of bone. (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).

View larger version (109K): [in a new window]   Figure 5.  Hyperostosis. Lateral radiograph of the right knee, obtained after deangulation osteotomy in a female patient aged 10 years, shows patellar and tibial hyperostosis (arrows), narrowing in the joint space, and irregular sclerosed surfaces in the patellofemoral and tibiofemoral compartments.

View larger version (124K): [in a new window]   Figure 6.  Hyperostosis. 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.

View larger version (67K): [in a new window]   Figure 7a.  Limb length discrepancy. 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.

View larger version (54K): [in a new window]   Figure 7b.  Limb length discrepancy. 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.

View larger version (66K): [in a new window]   Figure 8.  Asymmetric overgrowth. Axial T1-weighted MR image at the level of the upper thighs in a patient aged 7 years shows asymmetric overgrowth of the right femur and the muscles and subcutaneous tissues of the right thigh.

View larger version (111K): [in a new window]   Figure 9a.  Asymmetric overgrowth. 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.

View larger version (84K): [in a new window]   Figure 9b.  Asymmetric overgrowth. 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.

View larger version (127K): [in a new window]   Figure 10.  Abnormal calcaneus and cerebriform connective-tissue nevus. 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).

View larger version (128K): [in a new window]   Figure 11a.  Rapid progression of cervical spine overgrowth. (a) Right lateral radiograph obtained in a patient at age 6 years shows enlargement but relatively normal alignment of the cervical vertebral bodies. (b) Right lateral radiograph obtained at age 8 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.

View larger version (131K): [in a new window]   Figure 11b.  Rapid progression of cervical spine overgrowth. (a) Right lateral radiograph obtained in a patient at age 6 years shows enlargement but relatively normal alignment of the cervical vertebral bodies. (b) Right lateral radiograph obtained at age 8 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.

View larger version (117K): [in a new window]   Figure 12a.  Abnormal vertebral bodies. 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. Note also the rib asymmetry and thoracic scoliosis in a.

View larger version (116K): [in a new window]   Figure 12b.  Abnormal vertebral bodies. 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. Note also the rib asymmetry and thoracic scoliosis in a.

View larger version (84K): [in a new window]   Figure 13a.  Abnormal vertebral bodies. 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.

View larger version (84K): [in a new window]   Figure 13b.  Abnormal vertebral bodies. 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.

View larger version (79K): [in a new window]   Figure 14.  Abnormal vertebral bodies. Sagittal T1-weighted MR image of the thoracic spine in a patient aged 15 years shows marked variation in vertebral body shape and size, spondylosis at multiple levels, and posterior scalloping of several lower thoracic vertebrae, as well as thoracic kyphosis due to an abnormal midthoracic vertebral body (arrow).

View larger version (156K): [in a new window]   Figure 15a.  Skull abnormalities. 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).

View larger version (129K): [in a new window]   Figure 15b.  Skull abnormalities. 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).

View larger version (82K): [in a new window]   Figure 16a.  Skull abnormalities. (a) Axial CT image in a patient at age 3 months shows minimal calvarial thickening on the right side. (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.

View larger version (129K): [in a new window]   Figure 16b.  Skull abnormalities. (a) Axial CT image in a patient at age 3 months shows minimal calvarial thickening on the right side. (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.

View larger version (132K): [in a new window]   Figure 17.  Severe pes equinus. Lateral radiograph of the right foot in a patient aged 17 years shows marked enlargement of the distal tibia and fibula; deformity and abnormal flexion of the calcaneus in the dorsal direction (black arrowhead); enlargement of the distal portion of the proximal phalanx, proximal portion of the distal phalanx, and the sesamoid bones of the first ray (arrows); coarse trabeculation of the enlarged bones; and lobulation of the plantar skin (white arrowheads), a finding that indicates cerebriform connective-tissue nevus.

View larger version (117K): [in a new window]   Figure 18a.  Progressive skeletal changes. (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). (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.

View larger version (108K): [in a new window]   Figure 18b.  Progressive skeletal changes. (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). (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.

View larger version (114K): [in a new window]   Figure 18c.  Progressive skeletal changes. (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). (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.

View larger version (114K): [in a new window]   Figure 18d.  Progressive skeletal changes. (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). (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.

View larger version (115K): [in a new window]   Figure 19.  Fatty overgrowth. 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).

View larger version (69K): [in a new window]   Figure 20.  Fatty overgrowth. 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. The adipose overgrowth in this patient extended from the hand into the forearm.

View larger version (83K): [in a new window]   Figure 21a.  Fatty overgrowth. Axial T1-weighted MR images of the pelvis and upper thighs (a) and the upper to middle thighs (b) in a patient aged 20 years show asymmetric muscle development in the thighs, asymmetric focal increase of fatty tissue in the right buttock (* in a), diffuse increase of fatty tissue in the left thigh, and focal accumulation of fat anterior to the left distal femur (arrow in b).

View larger version (76K): [in a new window]   Figure 21b.  Fatty overgrowth. Axial T1-weighted MR images of the pelvis and upper thighs (a) and the upper to middle thighs (b) in a patient aged 20 years show asymmetric muscle development in the thighs, asymmetric focal increase of fatty tissue in the right buttock (* in a), diffuse increase of fatty tissue in the left thigh, and focal accumulation of fat anterior to the left distal femur (arrow in b).

View larger version (137K): [in a new window]   Figure 22a.  Lipoma and fatty infiltration of muscles. Axial (a) and sagittal (b) T1-weighted MR images of the thoracic spine in a 6-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).

View larger version (93K): [in a new window]   Figure 22b.  Lipoma and fatty infiltration of muscles. Axial (a) and sagittal (b) T1-weighted MR images of the thoracic spine in a 6-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).

View larger version (128K): [in a new window]   Figure 23a.  Fatty infiltration of muscles, and vascular malformations. (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. (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.

View larger version (121K): [in a new window]   Figure 23b.  Fatty infiltration of muscles, and vascular malformations. (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. (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.

View larger version (126K): [in a new window]   Figure 23c.  Fatty infiltration of muscles, and vascular malformations. (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. (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.

View larger version (103K): [in a new window]   Figure 24a.  Fatty infiltration of muscles and vascular malformations. 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.

View larger version (72K): [in a new window]   Figure 24b.  Fatty infiltration of muscles and vascular malformations. 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.

View larger version (138K): [in a new window]   Figure 25.  Venous malformations. 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.

View larger version (157K): [in a new window]   Figure 26a.  Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. (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. (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. (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. (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. (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 (*). (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.

View larger version (141K): [in a new window]   Figure 26b.  Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. (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. (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. (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. (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. (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 (*). (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.

View larger version (123K): [in a new window]   Figure 26c.  Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. (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. (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. (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. (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. (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 (*). (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.

View larger version (133K): [in a new window]   Figure 26d.  Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. (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. (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. (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. (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. (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 (*). (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.

View larger version (138K): [in a new window]   Figure 26e.  Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. (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. (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. (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. (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. (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 (*). (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.

View larger version (74K): [in a new window]   Figure 26f.  Lipomas, splenomegaly, cystic lung changes, and pulmonary embolism. (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. (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. (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. (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. (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 (*). (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.

View larger version (142K): [in a new window]   Figure 27.  Asymmetric megalencephaly. Axial T2-weighted MR image of the head of a patient aged 7 years shows broad gyration and diffuse enlargement of the right cerebral hemisphere, closed-lip schizencephaly beginning at the right lateral sulcus (arrows), and prominence of the Virchow-Robin spaces.