HEMIATROFIA FACIAL PROGRESIVA PDF

Skip to search form Skip to main content You are currently offline. Some features of the site may not work correctly. La hemiatrofia facial progresiva HFP o sindrome de Parry-Romberg es una entidad poco frecuente que se caracteriza por atrofia e hiperpigmentacion de la dermis con compromiso unilateral de la cara. La baja incidencia de esta patologia nos motivo a la presentacion, estudio y busqueda bibliografica de esta entidad clinica.

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Parry—Romberg syndrome PRS is a rare disease characterized by progressive shrinkage and degeneration of the tissues beneath the skin , usually on only one side of the face hemifacial atrophy but occasionally extending to other parts of the body. It has been reported in the literature as a possible consequence of sympathectomy. In addition to the connective tissue disease , the condition is sometimes accompanied by neurological, ocular and oral symptoms.

The range and severity of associated symptoms and findings are highly variable. Initial facial changes usually involve the area of the face covered by the temporal or buccinator muscles. This scar is referred to as a "coup de sabre" lesion because it resembles the scar of a wound made by a sabre, and is indistinguishable from the scar observed in frontal linear scleroderma.

Symptoms and physical findings usually become apparent during the first or early during the second decade of life. The average age of onset is nine years of age, [2] and the majority of individuals experience symptoms before 20 years of age.

The disease may progress for several years before eventually going into remission abruptly ceasing. Neurological abnormalities are common.

Enophthalmos recession of the eyeball within the orbit is the most common eye abnormality observed in Parry—Romberg syndrome. It is caused by a loss of subcutaneous tissue around the orbit. Other common findings include drooping of the eyelid ptosis , constriction of the pupil miosis , redness of the conjunctiva , and decreased sweating anhidrosis of the affected side of the face.

Collectively, these signs are referred to as Horner's syndrome. Other ocular abnormalities include ophthalmoplegia paralysis of one or more of the extraocular muscles and other types of strabismus , uveitis , and heterochromia of the iris. The tissues of the mouth, including the tongue, gingiva , teeth and soft palate are commonly involved in Parry—Romberg syndrome. The fact that some people affected with this disease have circulating antinuclear antibodies in their serum supports the theory that Parry—Romberg syndrome may be an autoimmune disease, specifically a variant of localized scleroderma.

However, there has also been at least one report of monozygotic twins in which only one of the twins was affected, casting doubt on this theory. Further, the National Organization for Rare Disorders has stated there is currently no evidence that Parry—Romberg syndrome is genetic or that it can be passed on to children.

It is likely that the disease results from different mechanisms in different people, with all of these factors potentially being involved. Diagnosis can be made solely on the basis of history and physical examination in people who present with only facial asymmetry. For those who report neurological symptoms such as migraine or seizures, MRI scan of the brain is the imaging modality of choice. A diagnostic lumbar puncture and serum test for autoantibodies may also be indicated in people who present with a seizure disorder of recent onset.

Medical management may involve immunosuppressive drugs such as methotrexate , corticosteroids , cyclophosphamide , and azathioprine. No randomized controlled trials have yet been conducted to evaluate such treatments, so the benefits have not been clearly established. Affected individuals may benefit from autologous fat transfer or fat grafts to restore a more normal contour to the face.

However, greater volume defects may require microsurgical reconstructive surgery which may involve the transfer of an island parascapular fasciocutaneous flap or a free flap from the groin, rectus abdominis muscle Transverse Rectus Abdominis Myocutaneous or "TRAM" flap or latissimus dorsi muscle to the face. Severe deformities may require additional procedures, such as pedicled temporal fascia flaps, cartilage grafts, bone grafts , orthognathic surgery , and bone distraction.

Parry—Romberg syndrome appears to occur randomly and for unknown reasons. Prevalence is higher in females than males, with a ratio of roughly The condition is observed on the left side of the face about as often as on the right side. The disease was first described in by Caleb Hillier Parry — , in a collection of his medical writings which were published posthumously by his son Charles Henry Parry — From Wikipedia, the free encyclopedia.

Disease characterized by degeneration of tissues beneath the skin. CT scan3D bone reconstruction of a year-old girl with Parry Romberg syndrome. Syndromes of the head and neck 4th ed. New York: Oxford University Press. Textbook of oral pathology. Archives of Neurology and Psychiatry. Practical Neurology. Journal of Medical Genetics. American Journal of Ophthalmology.

American Journal of Medical Genetics. National Organization for Rare Disorders. Retrieved News for referring physicians. Archived from the original on Transactions of the third international congress of plastic surgery International Congress Series No. Third International Congress of Plastic Surgery.

Amsterdam: Excerpta Medica. Parry, CH ed. Collections from the unpublished medical writings of the late Caleb Hillier Parry, M. London: Underwoods. Klinische ergebnisse in German. Lehrbuch der functionellen nervenkrankheiten auf physiologischer basis in German.

Berlin: Verlag von August Hirschwald. American Journal of Neuroradiology. ICD - 10 : G Orphanet : Nerve , nerve root, plexus. Carpal tunnel syndrome Ape hand deformity. Ulnar nerve entrapment Froment's sign Guyon's canal syndrome Ulnar claw. Radial neuropathy Wrist drop Cheiralgia paresthetica. Winged scapula Backpack palsy. Meralgia paraesthetica. Tarsal tunnel syndrome. Morton's neuroma. Trendelenburg's sign. Piriformis syndrome. Charcot—Marie—Tooth disease Dejerine—Sottas disease Refsum's disease Hereditary spastic paraplegia Hereditary neuropathy with liability to pressure palsy Familial amyloid neuropathy.

Alcoholic polyneuropathy. Oral and maxillofacial pathology K00—K06, K11—K14 , —, — Bednar's aphthae Cleft palate High-arched palate Palatal cysts of the newborn Inflammatory papillary hyperplasia Stomatitis nicotina Torus palatinus. Oral mucosa — Lining of mouth. Teeth pulp , dentin , enamel. Periodontium gingiva , periodontal ligament , cementum , alveolus — Gums and tooth-supporting structures. Cementicle Cementoblastoma Gigantiform Cementoma Eruption cyst Epulis Pyogenic granuloma Congenital epulis Gingival enlargement Gingival cyst of the adult Gingival cyst of the newborn Gingivitis Desquamative Granulomatous Plasma cell Hereditary gingival fibromatosis Hypercementosis Hypocementosis Linear gingival erythema Necrotizing periodontal diseases Acute necrotizing ulcerative gingivitis Pericoronitis Peri-implantitis Periodontal abscess Periodontal trauma Periodontitis Aggressive As a manifestation of systemic disease Chronic Perio-endo lesion Teething.

Periapical, mandibular and maxillary hard tissues — Bones of jaws. Agnathia Alveolar osteitis Buccal exostosis Cherubism Idiopathic osteosclerosis Mandibular fracture Microgenia Micrognathia Intraosseous cysts Odontogenic : periapical Dentigerous Buccal bifurcation Lateral periodontal Globulomaxillary Calcifying odontogenic Glandular odontogenic Non-odontogenic: Nasopalatine duct Median mandibular Median palatal Traumatic bone Osteoma Osteomyelitis Osteonecrosis Bisphosphonate-associated Neuralgia-inducing cavitational osteonecrosis Osteoradionecrosis Osteoporotic bone marrow defect Paget's disease of bone Periapical abscess Phoenix abscess Periapical periodontitis Stafne defect Torus mandibularis.

Temporomandibular joints , muscles of mastication and malocclusions — Jaw joints, chewing muscles and bite abnormalities. Salivary glands. Orofacial soft tissues — Soft tissues around the mouth. Eagle syndrome Hemifacial hypertrophy Facial hemiatrophy Oral manifestations of systemic disease. Categories : Connective tissue diseases Genetic disorders with OMIM but no gene Rare diseases Neurocutaneous conditions Syndromes affecting the skin Syndromes affecting the nervous system.

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The subcutaneous tissue and underlying facial muscles on the right side of the face are severely atrophic , while the left side is unaffected. Upper limb median nerve : Carpal tunnel syndrome Ape hand deformity.

Palate Bednar's aphthae Cleft palate High-arched palate Palatal cysts of the newborn Inflammatory papillary hyperplasia Stomatitis nicotina Torus palatinus. Periodontium gingiva , periodontal ligament , cementum , alveolus — Gums and tooth-supporting structures Cementicle Cementoblastoma Gigantiform Cementoma Eruption cyst Epulis Pyogenic granuloma Congenital epulis Gingival enlargement Gingival cyst of the adult Gingival cyst of the newborn Gingivitis Desquamative Granulomatous Plasma cell Hereditary gingival fibromatosis Hypercementosis Hypocementosis Linear gingival erythema Necrotizing periodontal diseases Acute necrotizing ulcerative gingivitis Pericoronitis Peri-implantitis Periodontal abscess Periodontal trauma Periodontitis Aggressive As a manifestation of systemic disease Chronic Perio-endo lesion Teething.

Periapical, mandibular and maxillary hard tissues — Bones of jaws Agnathia Alveolar osteitis Buccal exostosis Cherubism Idiopathic osteosclerosis Mandibular fracture Microgenia Micrognathia Intraosseous cysts Odontogenic : periapical Dentigerous Buccal bifurcation Lateral periodontal Globulomaxillary Calcifying odontogenic Glandular odontogenic Non-odontogenic: Nasopalatine duct Median mandibular Median palatal Traumatic bone Osteoma Osteomyelitis Osteonecrosis Bisphosphonate-associated Neuralgia-inducing cavitational osteonecrosis Osteoradionecrosis Osteoporotic bone marrow defect Paget's disease of bone Periapical abscess Phoenix abscess Periapical periodontitis Stafne defect Torus mandibularis.

Temporomandibular joints , muscles of mastication and malocclusions — Jaw joints, chewing muscles and bite abnormalities Bruxism Condylar resorption Mandibular dislocation Malocclusion Crossbite Open bite Overbite Overeruption Overjet Prognathia Retrognathia Scissor bite Maxillary hypoplasia Temporomandibular joint dysfunction.

Orofacial soft tissues — Soft tissues around the mouth Actinomycosis Angioedema Basal cell carcinoma Cutaneous sinus of dental origin Cystic hygroma Gnathophyma Ludwig's angina Macrostomia Melkersson—Rosenthal syndrome Microstomia Noma Oral Crohn's disease Orofacial granulomatosis Perioral dermatitis Pyostomatitis vegetans.

Other Eagle syndrome Hemifacial hypertrophy Facial hemiatrophy Oral manifestations of systemic disease.

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Parry–Romberg syndrome

Parry—Romberg syndrome PRS is a rare disease characterized by progressive shrinkage and degeneration of the tissues beneath the skin , usually on only one side of the face hemifacial atrophy but occasionally extending to other parts of the body. It has been reported in the literature as a possible consequence of sympathectomy. In addition to the connective tissue disease , the condition is sometimes accompanied by neurological, ocular and oral symptoms. The range and severity of associated symptoms and findings are highly variable. Initial facial changes usually involve the area of the face covered by the temporal or buccinator muscles. This scar is referred to as a "coup de sabre" lesion because it resembles the scar of a wound made by a sabre, and is indistinguishable from the scar observed in frontal linear scleroderma. Symptoms and physical findings usually become apparent during the first or early during the second decade of life.

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