1/10. 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 = connective (Clic here for more details about this article) |
2/10. Progressive facial hemiatrophy: report of five cases and biochemical analysis of connective tissue.Five cases of progressive facial hemiatrophy (PFH) are reported. A nonindurated depression on normal-colored skin was observed in the cheeks of 3 subjects, and 2 patients showed indurated, pigmented atrophic lesions associated with linear scleroderma or generalized morphea. Lipoatrophy with mild subcutaneous fibrosis was observed histologically in the patients with nonindurated depressions. In contrast, the patients with indurated lesions exhibited a marked dermal fibrosis and the disappearance of appendices in the dermis. When compared with unaffected skin used as a control, collagen and glycosaminoglycan contents were not different in diseased areas. However, the dermatan sulfate/hyaluronic acid ratio was increased 1.5- to 3.2-fold in PFH patients regardless of their clinical and histological differences. These results suggest that both types of PFH may be based on a similar connective tissue disorder.- - - - - - - - - - ranking = 5keywords = connective (Clic here for more details about this article) |
3/10. facial hemiatrophy--a report of 5 cases.Progassive hemifacial atrophy is an uncommon and poorly understood degenerative condition characterized by atrophic changes involving the entire one side of the face, head and associated structures and typically appearing during the growth spurts to young adulthood. The condition involves both soft tissues and bone of the affected side. Intraorally the tongue, lips and salivary glands may be involved. Developing teeth may also show incomplete root development deleyed eruption. The couse of the condition is obscure or unknown although speculation has considered trophic malfunction of the cervical sympathetic nervous system, a history of prior trauma has been documented in some cases, although other reports have considered a viral or borrelial infection. It is usualy sporadic but a few familial cases have been reported suggetsting a possible hereditary influence and many investigators believe it to be a localized from of scleroderma. Five cases of Hemifacial Atrophy (Parry Romberg Syndrom) has been reported and discused.- - - - - - - - - - ranking = 0.10799349001025keywords = soft (Clic here for more details about this article) |
4/10. Parascapular free flap and fat grafts: combined surgical methods in morphological restoration of hemifacial progressive atrophy.BACKGROUND: Progressive hemifacial atrophy, widely referred to as Parry-Romberg syndrome or Romberg's disease, is a rare pathologic process characterized by an acquired, idiopathic, self-limited, unilateral atrophy of the face, variably involving skin, subcutaneous tissues, fat, muscle and, less frequently, the underlying bone structures. methods: Restoration of contour and symmetry is the main challenge in cases of Parry-Romberg syndrome; there are several different reconstructive techniques directed at augmentation of deficient soft-tissue volume. The present study describes the authors' own experience with deepithelialized free parascapular flap transfer combined with Coleman's lipoinjection in treatment of four severe cases affected by Parry-Romberg syndrome. RESULTS: The free microvascular parascapular flap, harvested and transferred for facial contour reconstruction, provided large volumes of tissue at the location of the greatest contour defect. It also has proven useful in restoring atrophic tissues trophism. To achieve the finest reconstructive result, Coleman's lipoinjection technique was used for a modification of the free parascapular flap contour, to restore adjacent atrophic areas, and to treat the remaining small or moderate defects. CONCLUSIONS: Free parascapular flap transfer combined with Coleman's lipoinjection, according to the authors' results, is the procedure of choice for facial contour restoration. Together, they can provide an excellent reconstructive solution directed at restoration of facial morphology, in terms of natural appearance and long-lasting results, with minimal donor-site morbidity.- - - - - - - - - - ranking = 0.10799349001025keywords = soft (Clic here for more details about this article) |
5/10. The volume limitation of the galeal temporalis flap in facial augmentation.Galeal temporalis flaps based on the superficial temporal vessels have been used for facial augmentation and can be extended to the contralateral side beyond the midline in order to achieve maximum volume. In five patients, the volumes of extended galeal flaps were measured intraoperatively using a water displacement method. The calculated volume varied between 28 and 38 cm3. Experience with this flap showed satisfactory results with no complications; therefore, it is concluded that the extended galeal temporalis flap may be a first choice in the correction of facial soft tissue deficits less than 40 cm3. Clinical cases are presented.- - - - - - - - - - ranking = 0.10799349001025keywords = soft (Clic here for more details about this article) |
6/10. Free-fat injections for the correction of hemifacial atrophy.Three adult patients with long-standing hemifacial atrophy were treated with repeated free-fat injections at 4- to 8-week intervals. The longest follow-up study to date is 18 months, and following the expected postoperative resorption, no further loss of bulk of injected fat has been observed. On palpation, the feel of the fat is normal, and facial expression is also good. The relative ease of this procedure, which does not entail any scarring, appears to justify more widespread use of free-fat injections to restore facial soft-tissue depressions.- - - - - - - - - - ranking = 0.10799349001025keywords = soft (Clic here for more details about this article) |
7/10. Restoration of facial contour using free vascularized omental transfer.Three cases are described in which free omental transfer was utilized for facial soft tissue augmentation. Dissections in 30 cadavers confirmed the five patterns of vascular distribution, the diameter of the omental vessels, and the fat distribution within the greater omentum. The versatile omentum offers many advantages over the "deepithelialized" groin flap: long pedicle, larger vessel size, less bulk, and tissue that can be layered upon itself. Retention of septae along the natural facial contour lines has improved the placement and minimized the late migration of transferred omental tissue.- - - - - - - - - - ranking = 0.10799349001025keywords = soft (Clic here for more details about this article) |
8/10. Spontaneous carotid-jugular fistula and carotid dissection in a patient with multiple intracranial arachnoid cysts and hemifacial atrophy: a generalized connective tissue disorder? Case report.The authors report a case of a 45-year-old woman with pulsatile tinnitus who was found to have an unusual spontaneous fistula between the petrous internal carotid artery and internal jugular vein. The fistula resolved spontaneously, possibly related to daily manual compression of the ipsilateral common carotid artery. The patient also had a contralateral carotid artery dissection, multiple intracranial arachnoid cysts, and hemifacial atrophy. Her medical history was significant for easy bruisibility, abnormal scarring, and mitral valve prolapse. This association appears unique and may represent a previously underscribed generalized connective tissue disorder.- - - - - - - - - - ranking = 5keywords = connective (Clic here for more details about this article) |
9/10. Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry-Romberg disease): case report.Intracranial aneurysms are uncommon in children, and their presence often leads to suspicion of a systemic connective tissue disorder. We describe the case of a young male patient with progressive hemifacial atrophy (Parry-Romberg disease) and multiple intracranial aneurysms, a previously undescribed association, and propose that a neural crest defect may be the underlying abnormality in this patient. At age 5 years, the patient was treated for a giant aneurysm of the left cavernous carotid artery with carotid ligation in the neck and a superficial temporal artery-middle cerebral artery bypass. At age 12 years, the patient was similarly treated for a giant aneurysm of the right cavernous carotid artery, which had progressed from a previously noted minute dilatation at age 5 years, with carotid ligation and a superficial temporal artery-middle cerebral artery bypass. At age 21 years, the patient was endovascularly treated for a de novo saccular aneurysm of the left posterior cerebral artery at the P1-P2 junction and a fusiform aneurysm of the distal left posterior cerebral artery. Various studies have suggested that the facial dermis, the subcutaneous tissues, and the skeleton, as well as the tunica media of the cervicocephalic arteries, all arise from neural crest cells, and a disorder of neural crest migration might explain the constellation of findings in this patient.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
10/10. Parry-Romberg syndrome: follow-up imaging during suppressive therapy.Parry-Romberg syndrome is a poorly--understood disorder characterized by progressive hemifacial atrophy involving the skin, soft tissue, and bone. Involvement of the central nervous system with impairment in neurologic function occurs infrequently. We describe a child with this syndrome in whom central nervous system involvement, documented on serial MRI, played a prominent role. We have attempted to correlate the clinical course with the radiologic findings, and to determine the impact of prednisone and methotrexate on the intracranial lesions.- - - - - - - - - - ranking = 0.10799349001025keywords = soft (Clic here for more details about this article) |
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