1/6. New findings in the Parry-Romberg syndrome: a case report.PURPOSE: To describe further findings in the Parry-Romberg syndrome that might contribute towards a better understanding of the disease. DESIGN: Case report. methods: The clinical history of a patient with the Parry-Romberg syndrome was studied, and fluorangiography and echography were carried out. RESULTS: Clinical observation revealed classic hemifacial atrophy; fluorescein angiography showed telangiectasis, light staining of the retina, and leakage from the largest telangiectasis and the optic disc vessels; echography showed shrinkage of the eyeball and thinning of the extraocular muscles. CONCLUSIONS: enophthalmos in this disease is caused not only by progressive fat atrophy but also by shrinkage of the eyeball and thinning of the extraocular muscles; the shrinkage of the eyeball helps explain the presence of choroidal and retinal folding and hyperopia, confirming the primary involvement of connective tissue in this disease.- - - - - - - - - - ranking = 1keywords = observation (Clic here for more details about this article) |
2/6. Linear scleroderma associated with progressive brain atrophy.Linear scleroderma (LS) is characterized by scleroatrophic lesions affecting limbs and legs, unilaterally. Neurological involvement may be associated with ipsilateral facial and skull involvement in disorders referred to clinically as LS 'en coup de sabre', and Parry-Romberg syndrome.We report a child with LS presenting with a severe neurological disorder characterized by epilepsy, progressive mental deterioration and a rapid process of atrophy involving the ipsilateral cerebral hemisphere, but not associated with an overlying facial structure involvement. Functional brain studies showed a reduction in the diameter of the left internal carotid and of the left middle cerebral artery. Our observations suggest that neuroimaging studies should be considered in all patients with linear scleroderma, and such studies become necessary when neurological symptoms occur.- - - - - - - - - - ranking = 1keywords = observation (Clic here for more details about this article) |
3/6. Misdiagnosis of silent sinus syndrome.PURPOSE: To review cases of spontaneous enophthalmos erroneously diagnosed as silent sinus syndrome to identify other inflammatory disorders with a similar clinical presentation. methods: Retrospective observational case series. Chart reviews were performed covering encounters over a 5-year period, searching for patients referred to two physicians with an incorrect diagnosis of silent sinus syndrome. Only cases of acquired enophthalmos of reported nontraumatic or nonneoplastic cause were included. Particular attention was directed toward noting clinical features of upper eyelid position and periocular atrophy. RESULTS: Nineteen cases of erroneous referral for silent sinus syndrome were identified. Fourteen of these cases were due to tumor, trauma, congenital facial asymmetry, or diffuse facial lipodystrophy. Among the remaining cases, four were diagnosed as Parry-Romberg syndrome and one as linear scleroderma. CONCLUSIONS: Parry-Romberg syndrome and linear scleroderma must be distinguished from silent sinus syndrome as causes of inflammatory-mediated, spontaneous enophthalmos.- - - - - - - - - - ranking = 1keywords = observation (Clic here for more details about this article) |
4/6. MR of craniocerebral hemiatrophy.The magnetic resonance (MR) findings of three patients with cerebral hemiatrophy, the so-called Dyke-Davidoff-Masson syndrome, which is characterized by variable degrees of unilateral loss of cerebral volume and compensatory changes of the calvarium are presented. The condition was due to middle cerebral artery stroke in all patients. The pathologic alterations of cerebral tissue and the brainstem were reflected in detail on the MR studies. MR findings in addition to the primary vascular insult included prominence of the cortical sulci and perimesencephalic cistern in one subject with acquired infarction, but an absence of such generalized sulcal prominence in two cases of congenital infantile paralysis. Otherwise the secondary ipsilateral pathologic observations were quite similar in the patients with congenital and acquired ischemic disease and encompassed a unilaterally small cerebral hemisphere together with ipsilateral diploic calvarial expansion, elevation of the petrous bone and orbital roof, and hypoplasia/atrophy of the cerebral peduncle. Although computed tomography (CT) and MR are complimentary, it is felt that MR represents the imaging procedure of choice with respect to the assessment of the etiology and extent of cerebral parenchymal involvement in patients presenting with a clinical combination of congenital or early onset of seizures, hemiparesis/plegia, and/or craniofacial asymmetry.- - - - - - - - - - ranking = 1keywords = observation (Clic here for more details about this article) |
5/6. Free vascularized groin fat flap in hypoplasia and hemiatrophy of the face (a three years observation).Two female patients with facial malformation are presented: one with microsomia (17 years old) and the other (35 years old) with a very severe form of Rhomberg-Parry facial hemiatrophy. The defect in one side of the face was augmented in both cases by means of a free inguinal fat flap connected to the local vascular system with microvascular anastomoses. After a follow up of three years (first patient) and 18 months (second patient) no sign of resorption could be observed and the fat shows a normal histological structure. The arterial flow is proved with the Doppler flowmeter and in one patient with arteriography. The operative technique is described, the advantages and disadvantages in contrast to other procedures are discussed.- - - - - - - - - - ranking = 4keywords = observation (Clic here for more details about this article) |
6/6. Progressive hemifacial atrophy: ten-year observation of a case.The authors describe the case of a 14-year-old girl who experienced progressive hemifacial atrophy at the age of three-and-a-half years. The patient's early age at appearance of the condition resulted not only in atrophy of skin, subcutaneous tissue, and muscles, but also in very severe deformation of the right side of the face due to disorders in growth of cartilaginous and osseous tissue. Changes in her brain case and craniofacial skeleton included enophthalmos, underdevelopment of the eyelids, nose, hard plate, and dental process of the jaw including the premolar teeth, and a lack of the second molar. These changes caused an irregular line of bite. The differential diagnosis excluded hemifacial microsomy and sclerodermal hemifacial atrophy.- - - - - - - - - - ranking = 4keywords = observation (Clic here for more details about this article) |