進行性頭蓋骨骨折
WordNet
- interrupt, break, or destroy; "fracture the balance of power"
- breaking of hard tissue such as bone; "it was a nasty fracture"; "the break seems to have been caused by a fall" (同)break
- fracture a bone of; "I broke my foot while playing hockey" (同)break
- become fractured; "The tibia fractured from the blow of the iron pipe"
- break (a bone); "She broke her clavicle"
- break into pieces; "The pothole fractured a bolt on the axle"
- violate or abuse; "This writer really fractures the language"
- the act of cracking something (同)crack, cracking
- relating to or suitable for growth; "the growing season for corn"; "good growing weather"
- (electronics) the production of (semiconductor) crystals by slow crystallization from the molten state
- the bony skeleton of the head of vertebrates
PrepTutorEJDIC
- 〈U〉(特に)骨を折ること;骨を折った状熊 / 〈C〉割れ(裂け)目,(鉱物の)破砕面 / 〈足・腕などを〉‘を'骨折する;…‘を'砕く / 骨折する;砕ける
- 『頭がい骨』,しゃれこうべ / 《話》おつむ,頭
UpToDate Contents
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English Journal
- Epidermal nevus in association with some uncommon manifestations.
- Rostami P, Mahmoudi E, Sotoudeh A, Nakhaeimoghadam M, Lurkin I, Zwarthoff EC, Rezaei N1.Author information 1Nima Rezaei, MD, PhD, Children's Medical Center Hospital, Dr. Qarib St., Keshavarz Blvd., Tehran 14194, Iran; rezaei_nima@tums.ac.ir.AbstractEpidermal nevus syndrome (ENS) is a rare condition characterized by congenital epidermal nevi (EN) associated abnormalities of other organs including central nervous system (CNS), skeletal system, eyes, and oral cavity (1). ENS is divided into a group of distinct disorders, based on the associated epidermal nevus and genetic inheritance pattern. Organoid nevus could be seen in some ENS, including Schimmelpenning syndrome, phacomatosis pigmentokeratotica, nevus comedonicus syndrome, angora hair nevus syndrome, and Becker nevus syndrome (1). The manifestations of ENS could be due to genetic mosaicism in involved cells, leading to lethal autosomal dominant genotype (2). Several genetic defects have been shown to be responsible for ENS; among them, mutations in either FGFR3 (Fibroblast Growth Factor Receptor 3) or PIK3CA genes (phosphatidyl inositol 3-Kinase, Catalytic, Alpha) are seen in about half of EN patients (3). Herein, a girl with EN with various manifestations is presented, in whom several genes have been sequenced; all of them were normal An 8-month-old girl was referred to the Children's Medical Center, Pediatrics Center of Excellence in Iran, with aplasia cutis congenita on the occipital bone and congenital hemangioma on the occipital and left parietal bone (7 cm×4 cm). Linear epidermal nevus was also evident on the right side of her face and the front of her chest, associated with a macular pigmented congenital nevus on the left scapula. The patient was the first child to consanguine parents with no similar manifestations in the family. She had ambiguous genitalia and mislocated anus at birth; an adhesion band was detected between two labia major anteriorly. There was no male genital organ, while chromosomal analysis of peripheral lymphocytes revealed normal chromosomal pattern, 46, XX. The occipital aplasia cutis was removed at the first admission, while the microscopic result of pathologic analysis showed flattened and thin epidermis, edema at dermis, vascular proliferation, and scattered inflammatory cells. Fatty subcutaneous tissue was also seen. No adnexal structure was identified in the center of lesion. These findings were compatible with healed lesion of aplasia cutis. The lesion had irregular, grayish, fine nodularity in the middle portion macroscopically. The adhesion band was also relieved by cystoscopy. Right cheek skin biopsy showed skin tissue with the same dysmorphic folliculosebaceous apparatus in the dermis and hypertrichosis. Organoid nevus was considered. Excisional skin biopsy of the infraclavicular pigmented lesion was indicative of congenital compound nevus. Axial computed tomography (CT) scan of the brain without intravenous contrast media obtained at one month of age showed generalized hydrocephaly evident in the ventricular system, hypodensity in the left side of the cerebellum that was suspicious of mass lesion, and a defect in the posterior aspect of the right parietal bone with encephalocele. There was no significant pathologic finding in other organs of the brain parenchyma and skull. At two years of age, she was referred to our institution with chief complaint of growth retardation. Laboratory tests showed normal blood electrolytes and thyroid hormones, but slightly decreased phosphorus level (2-3 mg/dL). Due to suspected hypophosphatemic rickets, additional examinations and evaluations were performed. The left leg seemed to be by about 1 cm shorter on physical examination. X-ray images showed multiple bone fractures in the humerus, femur and tibia (Fig. 1). The height and weight were under 5 percentile. Bone age was 1.5 years. Serum analysis revealed low serum P (2.1 mg/dL), Cl (93 meq/L), and PTH (5 ng/L), normal serum Ca, along with normal urine analysis. Two years later, after resection of multiple scalp tumors, a 3.4 cm×3 cm congenital soft scalp tumor relapsed in the resection regions with a rapid growth in 2 months. The growing tumor was locally tender (in the absence of general headache), pulseless and located on the right parietal bone. There was also a skull tumor with a firm adhesion to adjacent dura. The hard tissue tumors as well as the soft tissue part were resected. Pathologic analysis diagnosed the lesion as a cavernous hemangioma penetrating the skin. Sections of the cutaneous part showed sclerosis and fibrosis in the dermis; hypodermis revealed neoplastic tissue composed of large vessels with cystically dilated lumina and thin walls. Some vessels showed thrombi, occasionally with organization noted in reticulin and trichrome stained section. Sections of bone specimen showed osteosclerosis and penetration of vascular neoplastic tissue in some areas. Magnetic resonance imaging (MRI) scan showed cystic lesion in the upper region of the midbrain (hydrocephalus). There was a lipoma in the craniocervical region post-cordally and a lipoma in the posterior C6-C7. Axial spiral CT scan of the brain without contrast medium with 3D also revealed mild supratentorial hydrocephalus. Atrophic changes were found in the right cerebellar hemisphere. Otherwise, the brain seemed unremarkable. In bone window and reconstructed images, a left parietal bony protrusion with adjacent subcutaneous soft tissue compound was seen. In order to identify the underlying gene mutation, DNA was extracted from blood and the FGFR3, PIK3CA, NRAS, HRAS and KRAS genes were analyzed for mutations using PCR-SnaPShot assays (4-6). No mutations were found in these genes. Table 1 depicts a list of the nucleotides that were analyzed by the assays. EN is a neurocutaneous lesion that can vary from a single lesion to a wide spectrum of systemic presentations associated with EN. Different classifications have been proposed to describe different phenotypes of ENS (1,7), but the case presented cannot be categorized in any of the types described. The patient suffered from a variety of common manifestations of ENS: cutaneous manifestations (reported frequency >30%) (8), including aplasia cutis congenita, hemangioma, hyperpigmented lesions and linear nevus; neurologic manifestations (reported frequency 50%-70%), including brain tumors and hydrocephalus; skeletal manifestations (reported frequency 50%-60%), including hypophosphatemic rickets and bone tumors; and ocular findings such as amblyopia (9,10). Schimmelpenning syndrome and phacomatosis pigmentokeratotica are two other diagnoses that could have been considered in this patient (1). However, some other common manifestations such as seizures (reported frequency 25%) (11) were absent in our case, but some rare conditions including genital ambiguous and mislocated anus were detected. We analyzed mutations that had been shown to be present in patients with ENS; however, in this patient, none of the previously described mutations was found. These findings could suggest either the presence of a mutation in an unknown gene being responsible for manifestations of this case or an undefined syndrome with manifestations resembling ENS.
- Acta dermatovenerologica Croatica : ADC.Acta Dermatovenerol Croat.2013 Dec;21(4):268-70.
- Epidermal nevus syndrome (ENS) is a rare condition characterized by congenital epidermal nevi (EN) associated abnormalities of other organs including central nervous system (CNS), skeletal system, eyes, and oral cavity (1). ENS is divided into a group of distinct disorders, based on the associated e
- PMID 24476618
- Early diagnosis and treatment of growing skull fracture.
- Wang X1, Li G, Li Q, You C.Author information 1Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, People's Republic of China.AbstractBACKGROUND: Growing skull fracture (GSF) is a rare complication of pediatric skull fractures and causes delayed-onset neurological deficits and cranial asymmetry. Early treatment is pivotal to prevent those complications. The aim of this study is to highlight the early diagnosis and treatment of GSFs.
- Neurology India.Neurol India.2013 Sep-Oct;61(5):497-500. doi: 10.4103/0028-3886.121918.
- BACKGROUND: Growing skull fracture (GSF) is a rare complication of pediatric skull fractures and causes delayed-onset neurological deficits and cranial asymmetry. Early treatment is pivotal to prevent those complications. The aim of this study is to highlight the early diagnosis and treatment of GSF
- PMID 24262452
- Growing skull fractures: guidelines for early diagnosis and effective operative management.
- Reddy DR.
- Neurology India.Neurol India.2013 Sep-Oct;61(5):455-6. doi: 10.4103/0028-3886.121907.
- PMID 24262444
Japanese Journal
- 乳幼児におけるまれな growing skull fracture の治療経験
- 田中 宏明,清川 兼輔,力丸 英明,高橋 長弘,坂本 有孝
- 日本頭蓋顎顔面外科学会誌 = Journal of the Japan Society of Cranio-Maxillo-Facial Surgery 27(1), 26-31, 2011-03-25
- NAID 10028200695
- 反復性細菌性髄膜炎によって明らかとなった進行性頭蓋底骨折の1例
- 前納 万里,中本 裕介,香田 翼,山本 哲也,横田 知之,木寺 えり子,港 敏則,吉田 真策,尾崎 雅宏,大井 静雄
- 日本小児科学会雑誌 114(12), 1905-1908, 2010-12-01
- NAID 10027695786
- 症例報告 進行性頭蓋底骨折の1例--発生メカニズムの考察とともに
- 長田 知恵子,勝田 俊郎,権丈 緑 [他]
- Brain and nerve 59(11), 1293-1297, 2007-11
- NAID 40015701277
Related Links
- growing skull fracture growing skull fracture A rare sequel to a cranial fracture, especially in the parietal region, in which the cranial fracture grows following lacerations involving the dura mater. They usually occur after severe head ...
- INDIAN 1195PEDIATRICS VOLUME 40__DECEMBER17, 2003 CASE REPORTS Discussion Growing skull fractures usually occur after severe head trauma during the first three years of the life (particularly infancy) and almost never ...
★リンクテーブル★
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- 関
- 骨折
- 小児は成人に比べ骨折の頻度が高い。
- 骨膜は厚く骨形成が旺盛、骨癒合が早い、骨膜が厚いために不完全骨折となることが多い(若木骨折)、自家矯正能が高い(屈曲変形は5歳までは30°、8歳までは20°の屈曲があっても完全に矯正されるが、回旋変形に対しては自家矯正力はほとんど働かない。長骨の短縮に対しても矯正能力があるが、過成長が長じやすい。骨幹端部の方が骨幹部よりも自家矯正能が高い)、靭帯損傷や脱臼はまれである(SOR.641改変)。
- ほとんどの骨折では保存的治療でよい。
- 骨折のうち多いものは上腕骨顆上骨折である。この骨折では骨片が回旋転位するため手術を行う。
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- 英
- growing skull fracture
- 同
- 拡大性頭蓋骨骨折 enlarging skull fracture、発育性頭蓋骨折、進行性拡大性頭蓋骨骨折
- 関
- 頭部外傷、小児頭部外傷
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- 同
- 頭蓋骨