The term Proteus syndrome was coined in 1983 to describe a disorder of skeletal, hamartoinatous and other mesodermal malformations. The syndrome was named after the Greek god Proteus, whose name means "the Polymorphous". Major manifestations are hemihypertrophy, macrodactyly, exostoses, epidermal nevi, characteristic cerebriform masses involving the plantar or palmar surfaces, a variety of subcutaneous masses and scoliosis. We report a 12-year-old boy with Proteus syndrome who had the unique features of this syndrome.
Child ; Exostoses ; Humans ; Male ; Mesoderm ; Nevus ; Numismatics ; Proteus Syndrome* ; Proteus* ; Scoliosis

Proteus syndrome is a rare condition that can be loosely categorized as a hamartomatous disorder. It is a complex disorder with multisystem involvement and great clinical variability. At this time, it is unknown how many individuals have Proteus syndrome, since not all cases of Proteus syndrome are documented. We do not know whether anesthetic problems in Proteus syndrome are common or not, but as there is a high incidence of cervical spine involvement, we anticipate significant anesthetic problems. We experienced such a case under general anesthesia for liposuction to lipomatosis in the posterior neck and right upper back, in a 18-year-old boy with Proteus syndrome. We report this case of Proteus syndrome and review the literature on this rare disease.
Adolescent ; Anesthesia, General ; Humans ; Incidence ; Lipectomy ; Lipomatosis ; Male ; Neck ; Proteus Syndrome* ; Proteus* ; Rare Diseases ; Spine

Proteus syndrome is a rare congenital hamartomatous condition with a variety of abnormalities affecting allthree germ layers including overgrowth of various parts of the body, hemihypertrophy, unusual skeletalmalformation, skin lesions, and various tumors. I describe the radiologic findings in a 12 year-old boy withProteus syndrome. Computed tomography and magnetic resonance imaging are very useful for the specific diagnosis.
Child ; Diagnosis ; Germ Layers ; Hamartoma ; Humans ; Magnetic Resonance Imaging ; Male ; Proteus Syndrome* ; Proteus* ; Skin

The Proteus syndrome is a recently described congenital harmatosis consisting of numerous clinical features of great variety. Mainly affected are the musculoskeletal system, primarily by hemihypertrophy, macrodactyly, exostoses and kyphoscoliosis, and the skin and the subcutaneous tissue, primarily by pigmented nevi and subcutaneous tumors. These findings are diagnostic features of Proteus syndrome. We report typical manifestations of Proteus syndrome in a 12-year-old boy with brief review of literature.
Child ; Exostoses ; Humans ; Male ; Musculoskeletal System ; Nevus, Pigmented ; Proteus Syndrome* ; Proteus* ; Skin ; Subcutaneous Tissue

Collagenomas, or connective tissue nevi of the collagen type, are hamartomatous growths of otherwise normal collagen. They are divided into two groups: the inherited form that contains familial cutaneous collagenoma and shagreen patches in tuberous sclerosis, and the acquired form that contains eruptive collagenoma and isolated collagenoma. Among them, isolated plantar collagenoma with a cerebriform appearance is a relatively rare type of connective tissue nevus. It has been described as one of the major skin findings in Proteus syndrome. We present an unusual case of acquired isolated plantar collagenoma presenting as cerebriform hyperplasia, which was not associated with Proteus syndrome.
Collagen ; Connective Tissue ; Hyperplasia ; Nevus ; Proteus Syndrome ; Skin ; Tuberous Sclerosis

Isolated collagenoma usually occurs on the plantar area and is frequently associated with Proteus syndrome. A few reports have described isolated collagenoma on extra-plantar areas such as palm, scalp, face, thigh and finger. Herein, we report a case of isolated collagenoma on the periungual area, which was confused with digital mucous cyst. A 26-year-old man visited our clinic with an asymptomatic, 0.8x0.7 cm-sized, firm, skin-colored nodule on the periungual area of the left fourth finger. The distal nail of the nodule showed a longitudinal groove. Histopathologic findings from periungual nodule showed thickened collagen bundles packing reticular dermis.
Adult ; Collagen ; Dermis ; Fingers ; Humans ; Nails ; Proteus Syndrome ; Scalp ; Thigh

Proteus syndrome (PS) is a mosaic disorder characterized by asymmetric overgrowth of a variety of tissues. Diagnostic criteria established in 1999 emphasized the mosaic distribution of lesions, progressive course, and disproportionate overgrowth. We present a case of proteus syndrome in a 12-year-old Filipino male with 9 year-history of enlargement of the left foot with soft, non-tender mass on the sole with a brain-like surface. Skin punch biopsy of the mass showed cerebriform connective tissue nevi which is pathognomonic of PS.

PS is a very rare disease with prevalence of less than 1 in 1,000,000 live births. Management of PS is extremely challenging, owing to the combination of the individuality of each case, the severity of the disease, and the risks of complications from procedures. A multidisciplinary clinical approach is strongly recommended to obtain the best possible management plans for individual patients.

Proteus syndrome (PS) is a rare congenital hamartomatous disorder with multisystem involvement. PS shows highly clinical variability due to overgrowth of the affected areas, and several features can make anesthetic management challenging. Little is known about the airway problem associated with anesthesia in PS patients. An 11-year-old girl with PS was scheduled for ear surgery under general anesthesia. She had features complicating intubation including facial asymmetry and disproportion, abnormal teeth, limitation of neck movement due to torticollis, and thoracolumbar scoliosis. This study reports on a case of deformed airway of a PS patient under fiberoptic bronchoscopy.
Anesthesia ; Anesthesia, General ; Bronchoscopy ; Child ; Ear ; Facial Asymmetry ; Female ; Humans ; Intubation ; Neck ; Proteus Syndrome* ; Proteus* ; Scoliosis ; Tooth ; Torticollis

Proteus syndrome is a congenital hamartomatous malformation that is characterized by a wide range of deformities, including craniofacial deformities. Proteus syndrome features partial gigantism and asymmetry of the limbs, plantar hyperplasia, hemangiomas, lipomas, lymphangiomas, varicosities, verrucous epidermal nevi, macrocephaly, cranial hyperostosis, and long bone overgrowth. We diagnosed Proteus syndrome in a male patient who visited our hospital with a chief complaint of limited mouth opening and report the case because we obtained a good healing outcome after treating the condition with a corrective osteotomy.]]>
Congenital Abnormalities ; Extremities ; Gigantism ; Hemangioma ; Humans ; Hyperostosis ; Hyperplasia ; Lipoma ; Lymphangioma ; Macrocephaly ; Male ; Mouth ; Nevus ; Osteotomy ; Proteus ; Proteus Syndrome

A 16-year-old Korean male patient presented with macrodactyly, hemihypertrophy of the face and extremities, plantar cerebriform hyperplasia, a subcutaneous mass of the left chest, macrocephaly and verrucous epidermal nevi. These findings are consistent with Proteus Syndrome. The clinical features, etiology, management, natural course and differential diagnosis of this case are discussed.
Adolescent ; Case Report ; Disease Progression ; Human ; Male ; Proteus Syndrome/diagnosis/etiology/*physiopathology/therapy