[Is progressive facial hemiatrophy a facial disease].
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Disease↗
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Results for “Facial Hemiatrophy”
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[Differential diagnosis of facial hemiatrophy & other hemilateral facial asymmetries].
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Diagnosis, Differential↗
[Stellate ganglion block therapy against progressive facial hemiatrophy].
A typical case with progressive facial hemiatrophy was treated with a new therapeutic trial, stellate ganglion block. The present case, a Japanese girl, suffered from progressive atrophy involving the soft tissue of the left buccal region, with onset at the age of 6 following a minor local trauma. She visited our hospital at the age of 9, and as soon as the diagnosis was made, left stellate ganglion block was initiated. Local injection of lidocaine was performed 53 times over the period of 1 year and 4 months. During this period of therapy and the subsequent follow-up period of 4 years, the state of atrophy remained unchanged and nonprogressive. Stellate ganglion block as a therapy against progressive facial hemiatrophy was considered to be worth further evaluation, although it was impossible to judge, based solely on our experience, whether the arrest of progression was attributable to the therapy. The rationale of this therapy was also discussed. It was based upon the assumption that atrophy may result from irritation of the cervical sympathetic nerve, one of the most popular theories regarding the pathomechanism of progressive facial hemiatrophy.
Autonomic Nerve Block↗
Intracranial findings in progressive facial hemiatrophy.
There have been infrequent reports of cerebral lesions associated with progressive facial hemiatrophy. Six children with progressive facial hemiatrophy were evaluated. Four were referred for evaluation of neurological deficits: 2 with seizures, one with left hemiparesis and one with learning problems. The remaining 2 patients had only facial hemiatrophy. Cranial computed tomography (CT) in 5 patients revealed the bony and soft tissue defects, but cerebral calcifications were seen in only 3 patients. Cranial magnetic resonance imaging (MRI) demonstrated areas of increased signal in the ipsilateral white matter on T2 weighted images in all 5 patients with upper facial atrophy. Ipsilateral cerebral lesions with progressive facial hemiatrophy may be more common than once believed. MRI sometimes reveals abnormalities of the white matter even in patients without neurologic symptoms, and may be more sensitive than CT in the diagnostic evaluation of patients with progressive facial hemiatrophy.
Adolescent↗
[Idiopathic and sclerodermic facial hemiatrophy with generalized myopathy. Clinical, electromyographic and histologic examinations of six patients].
Six patients with facial hemiatrophy (H.f) were thoroughly examined by clinical and laboratory investigations. Two were found to have idiopathic and four facial hemiatrophy due to different types of localized scleroderma. In all cases a generalized myopathy was present, demonstrated by electromyographical, histological, and biochemical means. In none of these cases a hereditary or neurological cause for the facial hemiatrophy was found. However, in two cases autoantibodies against nuclei and muscle were repeatedly obtained. No prolongation of sensory or pain chronaxy occurred in either the patients with sclerodermal or idiopathic facial hemiatrophy. These observations suggest that facial hemiatrophy can originate (a) in a localized scleroderma in the involved part of the face, (b) in an ipsilateral "sclérodermie en coup de sabre", or (c) in the coexistence of both. There is a close pathological and physiological correlation between idiopathic and sclerodermal facial hemiatrophy. In both forms of facial hemiatrophy the disease involves skeletal muscle tissue systematically. This myopathy is similar to that of progressive scleroderma, far exceeding the limited muscular involvement of localized scleroderma. Sclerodermal facial hemiatrophy can be associated with autoimmune phenomena.
Adipose Tissue↗
[Congenital facial hemiatrophy. Report of two pediatric cases].
In facial hemiatrophy two different types are known: the progressive acquired type, which is relatively more common, and the rare congenital non progressive form. Two children--both with congenital facial hemiatrophy--are presented, both showed a (horizontally and vertically) smaller lid fissure and antimongoloid configuration of the lid. In addition in one case we could observe: small corneal diameter, staphyloma posticum, paving stone degeneration of the retina, convergent strabismus, and vertical deviation, in the other case: ptosis, major astigmatism, and latent divergent strabismus.
Child↗
MR of brain involvement in progressive facial hemiatrophy (Romberg disease): reconsideration of a syndrome.
PURPOSE: To gain further insight into the pathogenesis of progressive facial hemiatrophy, a sporadic disease of unclear etiology characterized by shrinking and deformation of one side of the face. METHODS: We investigated possible brain involvement. MR of the head and face was performed in three female patients with progressive facial hemiatrophy. The central-nervous-system findings were correlated to a clinical protocol and a review of the literature. RESULTS: One patient with epilepsy had abnormal brain findings confined to the cerebral hemisphere homolateral to the facial hemiatrophy. These consisted of monoventricular enlargement, meningocortical dysmorphia, and white-matter changes. CONCLUSIONS: These MR findings, and corresponding neuroradiologic data disclosed by the review, indicate that homolateral hemiatrophy occasionally occurs in a subgroup of patients with progressive facial hemiatrophy. The MR features do not seem consistent with an underlying simple or nutritive atrophic process. We propose chronic localized meningoencephalitis with vascular involvement as a possible underlying cause of the occasional brain involvement in progressive facial hemiatrophy.
Adult↗
Otorhinolaryngological complications of progressive facial hemiatrophy (Romberg's disease).
Progressive facial hemiatrophy (PFH) is characterized by slowly progressive atrophy of subcutaneous tissue. Bone, muscles, nerves, the eye, and the brain may be affected by atrophy. Four patients suffering from various otorhinolaryngological complications of PFH or Romberg's disease are reported. Unilateral hearing loss could be located in the inner ear of one patient by audiologic examination. Localized bone destruction and disintegration of a portion of the anterior wall of the frontal sinus were observed in a patient after more than three decades. Marked shrinking of the homolateral parotid gland and homolateral masticatory spasm are reported as further otorhinolaryngological manifestations. The various complications of PFH call for close interdisciplinary cooperation.
Child↗
Progressive facial hemiatrophy (Parry-Romberg syndrome).
Three cases of progressive facial hemiatrophy are presented. In all three cases there is evidence of localised scleroderma or morphea in association with the facial hemiatrophy. This would seem to support the contention that the two disorders are closely related. In two cases, ocular complications are prominent and in one Raynaud's phenomena provide clear asymmetrical dilatation of the lateral ventricle suggest that there may be a central rather than a peripheral cause for the sympathetic overactivity.
Adult↗
Progressive facial hemiatrophy and epilepsy: a common underlying dysgenetic mechanism.
Progressive facial hemiatrophy (PFH), a rare disorder characterized by progressive and self-limited atrophy of the skin and the subcutaneous tissues, is often associated with epilepsy but the link between these two conditions is poorly understood. The cause of PFH remains unclear. We report four patients with PFH associated with partial epilepsy in whom brain MRI showed cerebral dysgenesis. The four patients (two men, two women; age range: 24 to 73 years) developed parasagittal PFH in their second decade. Seizures started before the age of 20 years in three patients and were refractory simple, or complex partial seizures. All the patients had focal MRI showing cortical dysgenesis, ipsilateral to PFH, consisting of cortex thickening, gyral effacement, and blurring of the white-gray interface. The underlying white matter was hyperintense on T2-weighted sequences, with nodular areas in two patients. These areas were stable over time, without contrast enhancement, and were consistent with the MRI characteristics of cystic encephalomalacia. These neuroradiologic features suggest a localized cerebral hemispheric defect of congenital origin. Because cells participating in the formation of the fronto-nasal bud derive from common progenitors with the cells that give rise to the cerebral hemisphere, we suggest that an early malformative process affecting one side of the rostral neural tube could underlie both cerebral dysgenesis and facial hemiatrophy.
Adult↗
[Romberg's syndrome. Progressive facial hemiatrophy].
Romberg's syndrome or progressive facial hemiatrophy is a rare affection that may nevertheless be encountered by stomatologists during the course of their professional life. Diagnosis is simple if clinical and radiographic signs are recognized, an effective iconography being of particular value. This report is concerned mainly with the latter feature, and also includes a description of the principal characteristics of the affection and an extensive bibliography.
Adolescent↗
Progressive facial hemiatrophy: abnormality of intracranial vasculature.
Progressive facial hemiatrophy (PFH) or Parry-Romberg syndrome is associated with ipsilateral brain lesions and neurologic symptoms. We describe a 35-year-old man with PFH and frequent hemiplegic migraine. On cerebral angiography, reversible vessel caliber changes were seen within the symptomatic hemisphere. An abnormality of the intracranial vasculature may be present in some patients with PFH and neurologic manifestations.
Adult↗
[Facial hemiatrophy with monolateral fibrosis of all extraocular muscles].
Cases of facial hemiatrophy manifest a variety of symptoms of monolateral atrophic processes of the skin, muscles, and bones. The symptoms are usually progressive and may also involve the ocular bulb and the orbit. In this paper the authors report the case of a 53-year-old woman with congenital fibrosis of all extraocular muscles and restricted motility of the enophthalmic bulb in all directions of gaze. Since, in addition, the ipsilateral side of the face is affected by discrete atrophy, this case has been classified as congenital, nonprogressive facial hemiatrophy. The findings are compared with those in other published cases.
Blepharoptosis↗
[Magnetic resonance of brain involvement in progressive facial hemiatrophy (Romberg's disease). Reconsideration of a syndrome].
UNLABELLED: Progressive facial hemiatrophy (PFH) is a sporadic disease of unclear etiology, characterized by shrinking and deformation of one side of the face. Reports and interpretations of CNS involvement in PFH, as deduced from the occurrence of seizures in some patients and documented by pneumoencephalography and CT findings in small series of patients, are contradictory. We examined three female patients with PFH, one with partial epilepsy, with the view to gaining further insight into the pathogenesis of the disease. METHODS: Routine MR examinations of the head and face were performed. RESULTS: Only the patient with epilepsy showed pathological findings, confined to the cerebral hemisphere homolateral to the facial hemiatrophy, and including monoventricular enlargement, meningo-cortical dysmorphia and white matter changes. CONCLUSIONS: The MR morphology, and corresponding neuroradiological and histopathological findings disclosed by a review of the literature, indicate that homolateral hemiatrophy is a typical finding for a subgroup of PFH patients, but do not support the model of a simple or nutritive atrophic process. We reconsider chronic localized meningo-encephalitis with vascular involvement as possible underlying cause of the occasional brain involvement in PFH.
Adult↗
["Saber-cut" scleroderma and Parry-Romberg facial hemiatrophy. Nosologic problems. Neurologic complications].
We report two cases of gradual facial hemiatrophy (Parry-Romberg syndrome). The first patient, an adolescent girl under treatment for hypothyroidism, presented with a very severe form combining advanced facial hemiatrophy, epilepsy with hemi-generalized seizures and hemiatrophy of the brain. The second patient was a girl who presented with localized scleroderma resembling a saber injury, homolateral cerebral atrophy and contralateral hemiparesis. Our two cases are evidence in support of a close relationship between saber injury-like scleroderma and the Parry-Romberg syndrome.
Atrophy↗
[Facial hemiatrophy, homolateral cervical linear scleroderma and thyroid disease].
A case of facial hemiatrophy and homolateral cervical band of scleroderma, complicated by hypothyroidism is reported. This case raises two problems: one is the problem of distinction between Romberg's disease and facial hemiatrophy due to a genuine localized scleroderma; the other concerns the relationship between localized scleroderma and dysthyroidism. The generalized scleroderma-dysthyroidism association has now been recognized, but the coexistence of thyroid disease and localized scleroderma has not yet been reported. Several pathogenetic hypotheses on this association are discussed.
Facial Dermatoses↗
[Cerebral manifestations of progressive facial hemiatrophy (Romberg's disease). Nuclear spin tomographic findings and literature review].
Progressive facial hemiatrophy (PFH) is a sporadic disease of unknown etiology. It is characterized by shrinking and deformation of one side of the face. Potential CNS involvement has repeatedly been suspected in some patients, but is still a matter of controversy. In this article we describe the clinical and MR imaging findings of the CNS in three female patients with PFH and present a comprehensive review of the literature. One of three PFH patients had partial epilepsy. MRI showed ventricular enlargement, white matter lesions, flattening of the cortical surface and meningeal adhesions homolateral to the facial hemiatrophy. Two other patients had completely normal intracranial findings. These findings confirm that cerebral hemiatrophy occurs in a subgroup of PFH patients. The MRI pattern, however, does not seem to be consistent with a simple atrophic or malnutritional process. We consider chronic localized meningoencephalitis with vascular involvement as a possible underlying mechanism for the occasional CNS involvement in PFH.
Adult↗
Neurologic abnormalities in two patients with facial hemiatrophy and sclerosis coexisting with morphea.
Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue, and the underlying bony structures. This syndrome has many features of linear scleroderma en coup de sabre but is distinguished by more extensive involvement of the lower face and by only slight cutaneous sclerosis. We describe two unusual children with both atrophic and sclerotic changes of half of the face coexisting with multiple plaques of typical morphea. Both children developed neurologic disturbances with cranial magnetic resonance imaging (MRI) abnormalities 2 years and 15 years, respectively, after the onset of cutaneous lesions. Thus considering that it may not be possible to correlate impairment in neurologic function and cutaneous disease, as illustrated by our patients, we emphasize the importance of an accurate follow-up.
Adolescent↗