phenylephrine-hydrochloride and Fibroma

Tricho-rhino-phalangeal syndrome (TRPS) is a rare autosomal dominant genetic disorder characterized by distinctive craniofacial and skeletal abnormalities, while non-ossifying fibroma (NOF) is a common benign bone tumour in children and adolescents. To date, no case of TRPS coexisting with NOF has been reported. This report presents a 12-year-old girl who had the characteristic features of tricho-rhino-phalangeal syndrome and non-ossifying fibroma with a fibula fracture.. A 12-year-old girl was admitted to the Department of Endocrinology and Diabetes for evaluation of brachydactyly and a right fibula fracture. Clinical examination revealed sparse scalp hair, a characteristic bulbous pear-shaped nose, and brachydactyly with significant shortening of the fourth metatarsal. Neither intellectual disability nor multiple exostoses were observed. Radiography of both hands showed brachydactyly and cone-shaped epiphyses of the middle phalanges of the digits of both hands with deviation of the phalangeal axis. Genetic analysis of TRPS1 identified a heterozygous germline sequence variant (p.Ala932Thr) in exon 6 in the girl and her father. Approximately 1 month before being admitted to our department, the girl experienced a minor fall and suffered a fracture of the proximal fibula in the right lower limb. The pathological cytological diagnosis of the osteolytic lesion was NOF. Ten months following the surgery, the lesion on the proximal fibula of the girl disappeared.. In conclusion, the present study is the first to report a rare case of NOF with a pathologic fracture in the fibula of a girl with TRPS. The identification of a missense mutation, (p.Ala932Thr), in exon 6 of TRPS1 in this kindred further suggested that the patient had type I TRPS and indicated that mutations in this exon may be correlated with more pronounced features of the syndrome. Radiological techniques and genetic analysis played key roles in the definitive diagnosis.

Topics: Adult; Base Sequence; Bone Neoplasms; Brachydactyly; Child; DNA-Binding Proteins; Exons; Female; Fibroma; Fibula; Fingers; Fractures, Spontaneous; Gene Expression; Hair Diseases; Humans; Langer-Giedion Syndrome; Male; Mutation; Neoplasms; Nose; Paternal Inheritance; Radiography; Repressor Proteins; Transcription Factors

Rhinosurgery in children and adolescents meets special requirements: Limited cooperation and reduced limits for the organ dose for ionizing radiological examinations aggravate diagnostics. On the other side, bone sutures and bone growth areas have to be respected intraoperatively, and regions of bones not yet calcified have to be distinguished from possible tumor infiltration. Computer assisted surgery (CAS) can help to identify these areas safely.. 5 patients, from the first to the 20 (th) year of life, suffering from tumors, malformation syndromes or therapy resistant nasal polyposis were treated with CAS in rhinosurgery.. In addition to radiological diagnostics, we performed 3D computed tomography of the skull for CAS. CAS enabled us to intraoperatively respect possible areas of bone growth, to identify regions with thin, not bonily developed cranial vault and to safely distinguish bone sutures from ethmoidal cells. CAS helped the surgeon to navigate in the not yet developed paranasal sinus system.. CAS is a useful complementary method in rhinosurgery of the developing skull of the child. In spite of the additional 3D computed tomography, the calculated organ dose of the ocular lense amounted to 5 millisievert, so a recommended maximal organ dose for the ocular lense of 15 millisievert was not exceeded.

Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Choanal Atresia; Craniofacial Dysostosis; Down Syndrome; Female; Fibroma; Humans; Imaging, Three-Dimensional; Infant; Male; Nasal Polyps; Nasopharyngeal Neoplasms; Nasopharynx; Nose; Nose Diseases; Sinusitis; Skull; Surgery, Computer-Assisted; Tomography, X-Ray Computed

The pathologic findings of a sclerotic fibroma of the skin may resemble those of other benign cutaneous lesions. In this report, the sequential pathologic changes of a recurrent sclerotic fibroma of the skin during a period of 7 years are documented. As the lesion became older, there was not only a decrease in cellularity, but also a progressive organization of the collagen bundles toward a "plywood-like" appearance.

Topics: Adult; Female; Fibroma; Humans; Immunohistochemistry; Neoplasm Recurrence, Local; Nose; Skin; Skin Neoplasms; Vimentin

Juvenile hyaline fibromatosis (JHF) is a rare disease of autosomal recessive heredity characterized by multiple subcutaneous nodules, hypertrophy of the gingiva, and flexural contracture of the joints including those of the cervical vertebrae and the mandibula. It has been pointed out that the clinical features render tracheal intubation profoundly difficult. We present a case of 6-year-old girl diagnosed as JHF and anesthetized for tumor resection and reconstruction of the naso-oral region. She was significantly small for her age weight 12.3 kg and lived on a wheel-chair because of flexion contracture of the extremities. However, the cervical spinal joints were not involved and the neck could be extended in our case. With extreme care and preparation, anesthetic induction and tracheal intubation were smoothly done although a tooth was damaged by the surgeon when gauze-packing was attempted into the pharynx before the operation. Anesthesia was maintained uneventfully and satisfactorily during the surgery under anesthesia with nitrous oxide, oxygen, sevoflurane, fentanyl and vecuronium. There was no prolongation of nor abnormal reaction to the effect of vecuronium observed in this patient.

Topics: Anesthesia, Inhalation; Child; Female; Fibroma; Humans; Mouth; Nose; Soft Tissue Neoplasms

Midfacial degloving can be characterized as an alternative surgical approach for exposing the bony structures of the midface. In combination with transient partial osteotomies the nasal cavities, the paranasal sinuses, the pterygopalatine fossa and the posterior parts of the anterior skull base are easily accessible. Using an intercartilaginous, a transseptal and a circumvestibular incision in the nose and a vestibular incision in the oral cavity the soft tissues of the upper face are mobilized and transposed cranially up to the infraorbital rim, the nasion and the lacrimal sac. Thus one can avoid scar formations in the face. In comparison with the common visible incisions in the face a bilateral exposure of midline structures is possible. The resected bone can be easily replaced and fixed with titanium miniplates for osteosynthesis. The soft tissue glove is replaced. A correct suture technique for readaptation especially in the nasal cavity is most important to avoid a circular stenosis of the nasal aperture. Between 1986 and 1991, 40 patients with various tumors (juvenile angiofibroma, inverted papilloma, esthesioneuroblastoma, squamous cell carcinoma of the maxillary sinus, benign tumors of the pterygopalatine fossa, clivus chordoma) underwent this procedure. Neoplasms and fractures of the anterior frontal skull base, the frontal sinus, the orbital cavity and the zygoma were less accessible due to the unsatisfactory exposure of these regions. Complications and side effects were rare. In five cases, a transient paresthesia of the infraorbital nerve and a facial edema were observed. In one case, a circular stenosis of the nasal aperture required a second plastic procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adenoma; Carcinoma; Fibroma; Hemangioma; Humans; Melanoma; Neoplasm Staging; Neurilemmoma; Neuroectodermal Tumors, Primitive, Peripheral; Nose; Nose Neoplasms; Paranasal Sinus Neoplasms; Paranasal Sinuses; Plasmacytoma; Postoperative Complications; Precancerous Conditions

An extremely rare case of fibromatosis in a 25-year-old Indian male is reported. The clinical examination and pre-operative findings were suggestive of a benign neoplasm, probably of neurogenic origin. The mass was well circumscribed and could be shelled out en masse. Histopathological examination showed it to be fibromatosis. An external rhinoplasty approach enabled a complete wide excision to be performed without any resulting cosmetic defect.

Topics: Adult; Fibroma; Humans; Male; Nose; Nose Neoplasms; Rhinoplasty

We report on a 85-year-old woman suffering from a fibrofolliculoma at the root of her nose. This benign tumor is very uncommon, and its histological findings are little known. Pathogenetically we suspect a self-contained interaction between the epithelial and mesodermal component of the pilary complex.

Topics: Aged; Connective Tissue; Female; Fibroma; Glycosaminoglycans; Humans; Nose; Skin; Skin Neoplasms

A case of juvenile hyaline fibromatosis in a ten-year-old Asian girl is presented. This autosomal recessively inherited condition has not previously been described in the radiological literature. This patient demonstrates well the characteristic radiological appearance. The literature of juvenile hyaline fibromatosis and of the other generalized fibromatoses is reviewed and the interrelationship is discussed.

Topics: Child; Chin; Ear; Female; Fibroma; Head and Neck Neoplasms; Humans; Nose; Radiography

Topics: Adult; Aged; Carcinoma, Squamous Cell; Connective Tissue; Facial Injuries; Fibroma; Fibromyalgia; Humans; Laryngeal Neoplasms; Larynx; Male; Nose; Skin

Topics: Adolescent; Adult; Child; Child, Preschool; Ethmoid Sinus; Female; Fibroma; Frontal Sinus; Giant Cell Tumors; Humans; Male; Maxillary Sinus; Middle Aged; Nasopharyngeal Neoplasms; Nasopharynx; Neoplasm Recurrence, Local; Neoplasms, Connective Tissue; Nose; Nose Neoplasms; Ossification, Heterotopic; Osteoma; Osteoma, Osteoid; Osteosarcoma; Paranasal Sinus Neoplasms; Paranasal Sinuses; Sphenoid Sinus

Topics: Adult; Blood Vessels; Cold Temperature; Dilatation; Estrogens; Female; Fibroma; Granuloma; Humans; Nasal Mucosa; Nasopharyngeal Diseases; Nose; Pregnancy; Pregnancy Complications

Topics: Adolescent; Black People; Child; Congenital Abnormalities; Ear, External; Fibroma; Fibromatosis, Gingival; Genetics, Medical; Gingiva; Humans; Hyperplasia; Hypertrophy; Nose; Pathology; Radiography; Skeleton; Splenomegaly; West Indies

Topics: Fibroma; Humans; Medical Records; Nasal Cavity; Neoplasms; Nose; Osteoma; Paranasal Sinus Neoplasms; Paranasal Sinuses; Telangiectasis

Topics: Fibroma; Humans; Nasopharyngeal Neoplasms; Nose; Pharynx

Topics: Fibroma; Humans; Hypotension; Hypotension, Controlled; Nasopharyngeal Neoplasms; Nose; Pharynx

Topics: Fibroma; Humans; Nasopharyngeal Neoplasms; Nose; Pharynx

Topics: Fibroma; Humans; Nasopharynx; Nose; Pharynx

Topics: Fibroma; Humans; Neoplasms; Nose; Pharynx

Topics: Fibroma; Humans; Neoplasms; Nose; Sphenoid Sinus

Topics: Fibroma; Humans; Neoplasms; Nose; Pharyngeal Neoplasms; Pharynx