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Demographic and clinical findings of patients with mucocutaneous morphea have not been well characterized, to our knowledge.To determine the demographic and clinical characteristics of morphea patients with mucocutaneous lesions who were enrolled in the Morphea in Adults and Children cohort.Cross-sectional study of 735 patients in the Morphea in Adults and Children cohort from 2007 to 2018.A total of 4.6% of linear morphea patients had oral involvement versus 2.4% among the entire cohort, whereas 10.3% of generalized morphea patients had genital involvement versus 3.7% among the entire cohort. Patients with genital lesions were older at disease onset than those with oral morphea (57 versus 11.5 years; P .001) and had more frequent extragenital lichen sclerosus et atrophicus (59.2% versus 5.6%; P = .004).Selection bias and limited number of affected subjects.Oral morphea lesions predominate in younger patients with facial linear morphea, whereas genital lesions predominate in postmenopausal women with overlying extragenital lichen sclerosus et atrophicus.

Highlights • Progressive haemifacial atrophy, also known as Parry-Romberg syndrome. • Lipofilling provides an option for voluminous flaps, improving skin quality and preserving the natural contours of the face. • Fat reinjection is a simple, efficient and reproducible procedure. • Physical appearance and psychological well-being should be considered as a part of the general management., Introduction The aim of this paper is to present our experience using autologous fat transplantation for facial augmentation and to evaluate the effectiveness of lipofilling in different regions of the face for the reconstruction and aesthetic improvement of the facial contouring. Case presentation Data in term to the age, gender distribution, harvest and injection sites, degree of satisfaction with post-surgical facial, and complications were recorded. Discussion Progressive haemifacial atrophy, also Known as Parry Romberg's disease, is a common craniofacial disease that conventionally affects the subcutaneous tissue on one side of the face. We report here a series of 27cases of hemifacial atrophy (Parry-Romberg syndrome), admitted to our department, and treated with lipofilling in conformity with Coleman's guidelines. The volume of fat grafting was assessed by an attempt to reach symmetry with the controlateral side within a single procedure. Conclusion All patients claimed that they were satisfied or very satisfied with the aesthetic results and affirmed that it had a positive psychological impact on their daily life.

Epilepsy represents an essential component of Parry Romberg syndrome (PRS). This study aimed to identify clinical factors that influence the development of epilepsy and drug-resistant epilepsy (DRE) in PRS.We retrospectively reviewed the medical records of eighty patients with PRS. Data including the age of onset for PRS, history of seizures, use and timing of immunotherapy, antiseizure medication use, and EEG and brain imaging findings were reviewed. For comparison with the patients with epilepsy (PRSe+) group, we selected 18 age and sex-matched controls from the patient without epilepsy (PRSe-) cohort using propensity score matching.Eighteen (22.5%) had epilepsy: 12 were female, and the median age was 14.5 years (range = 6-48 years). Eleven patients developed DRE. The median latency between the onset of cutaneous manifestations and diagnosis and timing and use of immunotherapy was similar between the PRSe + and PRSe- groups. Intracranial abnormalities were commonly seen in the PRSe + group (16 vs. 2, p 0.01). White matter disease and ipsilateral atrophy were common among the PRSe + group. Timing and use of immunotherapy, epileptiform discharges, and brain imaging abnormalities did not differ between those with DRE and without.The presence and degree of severity of ipsilateral brain abnormalities are risk factors for the development of epilepsy in PRS but not factors in predicting drug resistance. The timing of immunotherapy did not influence the development of PRSe + or DRE. Prospective studies are needed to identify biomarkers for epilepsy and assess the role of immunotherapy on seizure outcomes in PRSe + .

Parry-Romberg syndrome (PRS) and linear sclerosis en coup de sabre (LScs) are rare, related, autoimmune conditions of focal atrophy and sclerosis of head and face which are associated with the development of focal epilepsy. The scarcity of PRS and LScs cases has made an evidence-based approach to optimal treatment of seizures difficult. Here we present a large systematic review of the literature evaluating 137 cases of PRS or LScs, as well as three new cases with epilepsy that span the spectrum of severity, treatments, and outcomes in these syndromes. Analysis showed that intracranial abnormalities and epileptic foci localized ipsilateral to the external (skin, eye, mouth) manifestations by imaging or EEG in 92% and 80% of cases, respectively. Epilepsy developed before external abnormalities in 19% of cases and after external disease onset in 66% of cases, with decreasing risk the further from the start of external symptoms. We found that over half of individuals affected may achieve seizure freedom with anti-seizure medications (ASMs) alone or in combination with immunomodulatory therapy (IMT), while a smaller number of individuals benefitted from epilepsy surgery. Although analysis of case reports has the risk of bias or omission, this is currently the best source of clinical information on epilepsy in PRS/LScs-spectrum disease. The paucity of higher quality information requires improved case identification and tracking. Toward this effort, all data have been deposited in a Synapse.org database for case collection with the potential for international collaboration.

Progressive hemifacial atrophy (PHA) is a rare disorder characterized by slow, unilateral atrophy of the soft tissues and bones of the craniofacial region. The defect becomes more pronounced with age, leading to esthetic and functional deficits. However, the proper timing and method of surgical reconstruction are still debated. The correction of this defect markedly influencing the quality of life of the patient can be achieved with less invasive to more invasive surgical approaches. A 21-year-old female patient with hemifacial atrophy and extensive alopecia presented to our clinic. Considering the body type and the expectations of the patient, a profunda artery perforator flap was applied for the reconstruction and esthetic improvement of the facial region. The facial asymmetry attenuated after the reconvalescence period. This case shows that in the up-to-date surgical treatment of severe PHA, the use of microvascular free flaps may provide a better approach when trying to achieve an acceptable esthetic result. This is the first time that a profunda artery perforator flap was used to restore facial asymmetry caused by PHA.

Summary Background Patients with congenital or acquired deformities always need fat graft at least twice to remodel facial contour. The purpose of this study was to objectively evaluate the outcomes of the first and second fat grafts for Romberg syndrome by using three-dimensional (3D) volumetric analysis. Method A retrospective study was conducted using the medical records of 13 patients with Romberg disease who were treated with fat graft twice for facial filling. Patient and surgeon satisfactions were evaluated by comparing the preoperative and postoperative two-dimensional (2D) photographs. The preoperative and postoperative 3D images were analyzed with professional analysis software to calculate the volume discrepancy between them. Result According to the 2D photographs, both patients and the surgeon were more satisfied with the cosmetic results of the second fat graft. There was no significant statistical difference in the injection volumes between the two surgeries (p = 0.1); however, the survival rate of the second fat graft (75.1% ± 7.7%) was much higher than that of the first (43.3% ± 11.1%) with a significant difference (p Conclusion In this study, 3D images were used to objectively evaluate the survival rate of fat graft. The results showed that the second fat graft without any sophisticated procedures could acquire better cosmetic result and volume retention in Romberg syndrome. This study result could serve as a guide for clinical surgeons to forecast the outcome of sequential fat graft for patients with congenital or acquired deformities.

Parry-Romberg syndrome is a rare disease characterized by slowly progressive atrophy affecting facial subcutaneous tissues, including the underlying muscles and osteocartilaginous structures. Various periocular, ocular, and neuro-ophthalmological manifestations have been described in Parry-Romberg syndrome. The most common periocular disorders include enophthalmos, eyelid, and orbit alterations. The most frequent ocular disorders include corneal and retinal changes, and the most common neuro-ophthalmological disorders involve optic nerve, ocular motor and pupillary dysfunction. Besides the characteristic facial abnormalities, systemic manifestations may occur, including neurologic, dermatologic, cardiac, endocrine, infectious, orthodontic, and maxillofacial disorders. So far, mainly brief case reports describe these ophthalmological findings. Therefore, we summarize the ocular, periocular, and neuro-ophthalmological findings in detail, describe diagnostic modalities, and outline therapeutic options.

Objective This study aimed to discuss a case of a patient with a known diagnosis of Parry–Romberg syndrome (PRS) presenting with ischemic stroke, the second such reported case. Background PRS is a rare genetic disorder with progressive hemifacial atrophy, which usually presents within the first 2 decades of life. Neurologic manifestations include trigeminal neuralgia with associated deafness, hemifacial pain with associated migraine headaches, seizures, movement disorders, and neuropsychiatric symptoms. Many patients have elevated antinuclear antibody (ANA) titers. However, stroke is uncommon. Case description A 34-year-old right-handed woman, diagnosed with PRS at age 15, presented with right-sided weakness on waking up. Brain magnetic resonance imaging revealed a small infarct of the posterior limb of the left internal capsule. Vessel imaging revealed an aberrant right subclavian artery. Atrophy of the right-sided muscles of mastication is consistent with her known diagnosis of right-sided PRS. Stroke workup revealed a patent foramen ovale; however, no evidence of deep venous thrombosis was found. Hypercoagulability workup revealed an elevated ANA. The cause of stroke in this patient with PRS remains unclear, as she has no known risk factors. Conclusion It is possible that elevated inflammatory markers associated with PRS may cause a proinflammatory state and predispose patients to small-vessel vasculopathy. It is important to note the association between PRS and ischemic stroke.

Treatment of hemifacial atrophy presents a challenge for reconstructive surgeons. Previous studies have described numerous methods for the correction of facial asymmetry. We present our experience with treatment of hemifacial atrophy using a microsurgical anterolateral thigh adipofascial flap procedure and other adjunctive measures. This method is similar to that used for the free anterolateral thigh flap, but only the deep fascia of the anterolateral thigh and subcutaneous fatty tissue above the fascia were harvested. This flap procedure was used in 32 patients with moderate or severe hemifacial atrophy. In the first stage, the anterolateral thigh adipofascial flap procedure was used in all the patients, of whom eight accepted a porous polyethylene implant along with the anterolateral thigh adipofascial flap to reconstruct the skeleton. In the second stage, ancillary procedures including porous polyethylene implantation, liposuction debulking, fat injection and flap re-suspension were performed to refine the outcome in 28 patients. The anterolateral thigh adipofascial flap is advantageous in that it can provide a reliable vascular pedicle with relatively thin, pliable soft tissue and direct primary closure of the donor site.

Objective Parry–Romberg syndrome, popularly known as progressive hemifacial atrophy, is a rare and complicated craniofacial deformity characterized by idiopathic and progressive atrophy of patient's face with compromised facial aesthetics and functions. In the present study, we performed a retrospective review on the vascularised serratus anterior muscle flap transfer for the treatment of the hemifacial atrophy in 4 consecutive Chinese patients with Parry–Romberg syndrome. Methods The serratus anterior muscle flaps were harvested and transferred to correct the facial defects together with simultaneous nerve anastomosis between the long thoracic nerve and the ipsilateral cervical branch of facial nerve in the patients with Parry–Romberg syndrome. The postoperative results from patients’ own perception of improvement of facial profile and symmetry were also evaluated. Results In this patient series, both of the vessels and nerves of all flaps were anastomosed successfully in the recipient facial regions without any microvascular failures. Satisfactory facial contour and favorable symmetry were achieved with minimal donor-site morbidity, complications, recurrence and little resorption in surgical reconstruction of hemifacial atrophy during our follow-up period. Conclusions Our results suggested that vascularised serratus anterior muscle flap transfer combined with simultaneous nerve anastomosis is a reliable and effective treatment of choice that offers satisfactory results in the surgical correction of facial atrophy associated with Parry–Romberg syndrome.

Facial hemiatrophy is a typical manifestation of Parry-Romberg syndrome, characterized by a slow progressive atrophy that appears in early stages of life, primarily affecting the subcutaneous tissue and subjacent fat on 1 side of the face. We describe the case of a 42-year-old female patient with stabilized moderate facial hemiatrophy on the left side of the face, successfully treated with a 2-stage autologous fat transplant and the use of subcutaneous tunnels among the musculature for the placement of the graft. We also describe the principal forms of correcting facial asymmetry in patients with Parry-Romberg syndrome and demonstrate that an autologous fat graft provides good results in the correction of this deformity, with improved esthetics and patient satisfaction.

Benign tumors of the peripheral nerve sheath, termed neurofibromas, are the hallmark feature of neurofibromatosis type 1 (NF1). These tumors can result in hypertrophy of a limb or another anatomic region. Hemifacial hypertrophy due to an underlying neurofibroma is a typical manifestation of NF1 in young children although the overall frequency of facial involvement is low. We retrospectively studied all patients, which were referred to our outpatient clinic because of hemihypertrophy or swelling of the face for initially unknown reason with a final diagnosis of NF1. A total number of six patients were identified. Clinical and radiological characteristics of these patients were analyzed. In all patients, diagnosis of NF1 could be established based on the typical clinical criteria. However, despite other typical NF1-associated features (e.g. multiple café-au-lait spots) diagnosis of a plexiform neurofibroma as underlying cause for the hemifacial hypertrophy was significantly delayed in all patients. MRI scans were misinterpreted in all of the cases as lymphangioma because plexiform neurofibromas can resemble mesenchymal tumors or lymphangiomas. NF1 has to be considered in the differential diagnosis of hemifacial hypertrophy. A thorough clinical examination of affected patients should focus on typical disease-defining features. Early diagnosis of NF1 can prevent unnecessary treatment at least in some patients.

Background Progressive facial hemiatrophy (PFH) is characterized by a slowly progressive atrophy of soft tissues and in some cases bony structures. Coexisting features of localized scleroderma (LS) are commonly observed, indicating the close nature of both disorders. Objective We sought to investigate clinical, serological, and radiographic findings in PFH from 278 patients with LS and to discuss the relationship to linear scleroderma en coup de sabre (LSCS). Method A total of 12 patients with PFH were retrospectively evaluated on the basis of clinical, serological, and radiographic findings. Results Five patients (42%) presented with complete PFH, and 7 patients (58%) with partial PFH involving either cheek or forehead. Five of the patients (42%) had a coexisting LSCS lesion, and 3 of them (25%) had concomitant LS of the trunk. Intraoral involvement was present in 6 cases. Neurological involvement was common, in particular, epileptic seizures. Serologic investigations showed neither evidence for infection with Borrelia burgdorferi nor any other indication of underlying systemic immunological disorders. Limitations There were a relatively small number of patients in a retrospective study. Conclusion The reported cases suggest a close relationship between PFH and LSCS. PFH might appear as two different subtypes, one involving cutaneous structures and presenting with clinical features similar to LSCS, one being strictly restricted to subcutaneous structures, primarily affecting the cheek area. The manifold clinical features of central nervous system involvement indicate the pathogenetic importance of neurological involvement in the development of PFH. Magnetic resonance imaging should be included in the tools of standard diagnostic procedures in patients with PFH. The etiologic relevance of autoimmunity as well as preceding trauma should be investigated in larger collective studies.

Background Parry-Romberg syndrome or progressive facial hemiatrophy is a rare clinical entity of an unknown etiology. We present the case of a 56-year-old woman with Parry-Romberg syndrome and a giant internal carotid artery aneurysm. Case Description The case was a 56-year-old woman with Parry-Romberg syndrome and a giant internal carotid artery aneurysm. Its association was extremely rare. Our report and some other reports of Parry-Romberg syndrome with cerebral aneurysms suggest the hypothesis that Parry-Romberg syndrome could be related to some inflammatory causes such as an autoimmune disease. Conclusion We present the case of a 56-year-old woman with Parry-Romberg syndrome and a giant internal carotid artery aneurysm. Its association is extremely rare and will support the hypothesis that Parry-Romberg syndrome could be related to some inflammatory causes such as an autoimmune disease.

Progressive facial hemiatrophy (PFH), Parry–Romberg syndrome, is a rare disorder frequently associated with epilepsy. We describe a 28-year-old man who had PFH and partial epilepsy that was easily controlled with antiepileptic drugs. In accordance with this patient’s benign course of seizures, the cortical silent period was prolonged in the symptomatic hemisphere. This finding may represent compensatory interictal mechanisms in epilepsy.

Aim: To gain further insight into the pathogenesis of Parry–Romberg syndrome, a sporadic disease of unknown aetiology characterized by progressive wasting of one side of the face. Method: Cranial MRI was performed in 10 patients with Parry–Romberg syndrome. The central nervous system findings are correlated to clinical findings and a review of the literature. Results: Three patients with a history of migraine had abnormal brain findings confined to the cerebral hemisphere ipsilateral to the facial hemiatrophy. Two patients without CNS symptoms had intracranial changes, one ipsilateral, the other both ipsilateral and contralateral to the facial hemiatrophy, on MRI. These changes consisted of either intracerebral atrophy or white matter hyperintensity. Five patients without CNS symptoms had no pathological intracranial MRI appearances. Conclusion: A significant number of patients with Parry–Romberg syndrome may have underlying brain involvement. These findings are consistent with previous reports.

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.

Department of Neurology, University Hospital Erlangen, Schwabachanlage 6, 91054 Erlangen, Germany Department of Neuroradiology, University Hospital Erlangen, Schwabachanlage 6, 91054 Erlangen, Germany Department of Neuropathology, University Hospital Erlangen, Schwabachanlage 6, 91054 Erlangen, Germany Department of Neurosurgery, University Hospital Erlangen, Schwabachanlage 6, 91054 Erlangen, Germany Department of Epileptology, University of Bonn Medical Centre, Germany

A pediatric patient with diagnosis of Parry-Romberg syndrome, or progressive hemifacial atrophy, presented with new-onset unilateral alien hand syndrome, which was attributed to focal progressive atrophy and gliosis in the contralateral thalamus observed on serial neuroimaging. This case illustrates not only the clinical-radiographic correlation between alien hand syndrome and contralateral thalamic lesion, but also involvement of deep gray structure in Parry-Romberg syndrome causing a rare movement disorder.

We present a case in which the patient had both bilateral shortening of the roots, and hemifacial atrophy. As far as we know, this combination has not been described before.

Progressive facial hemiatrophy or Parry-Romberg syndrome is characterised by slowly progressive atrophy of one side of the face, primarily involving the subcutaneous tissue and fat. The restoration of contour and symmetry is the main challenge in patients affected by this syndrome. We describe the case of a patient with stabilised mild hemifacial atrophy, successfully treated by autologous fat transplantation. Although two- or three-stage fat grafting may be needed in order to accomplish the desired result, in this specific case it was achieved in a single procedure. An accurate soft tissue substitution with creation of multiple subcutaneous and intramuscular tunnels for lipografting ensured adequate blood supply of the transplanted fat. The aesthetic improvement and the patient satisfaction show that autologous fat transplantation could be a safe technique for the correction of the mild Parry-Romberg syndrome.

Parry-Romberg is a rare syndrome of unknown origin, characterized by hemiatrophy of the face including subcutaneous tissue, skeletal muscle, and bones, along with various ocular and central nervous system abnormalities. Some investigators consider that injury to the sympathetic fibers of the trigeminal nerve is a cause for evolution of this syndrome. Various central nervous system symptoms have been reported in correlation with the syndrome, including epilepsy and hemiparesis. These symptoms were related to ipsilateral (or, less frequently, contralateral) facial lesions, and in a few case reports were consistent with Rasmussen's encephalitis-like lesions. Many clinical features overlap between facial linear scleroderma and en coup de sabre syndrome, which is characterized by localized inflammation leading to atrophy of the skin and subcutaneous tissues mainly on one side of the face; such overlap can lead to confusion in diagnosis. Furthermore, central nervous system involvement has been reported in en coup de sabre syndrome, leading to further misdiagnosis. The distinction between these two disorders is much disputed. Detailed here is the case of a child who had been diagnosed with en coup de sabre syndrome presenting with severe status migrainosus. Subsequent pathologic clinical, and neuroimaging findings led to a diagnosis of Parry-Romberg syndrome. This diagnosis is set in the context of the similarities, contradictions, and growing confusion between the two syndromes.

Parry-Romberg syndrome (PRS) is a rare clinical entity of unknown etiology, generally characterized by a slow and progressive atrophy that affects 1 side of the face. A variety of neurologic abnormalities have been shown to coexist with PRS. However, few studies regarding pyramidal tract involvement in this disorder have been reported. We report a unique case, in which the patient presented with bilateral pyramidal tract insult and an unusual sequence of disease onset and progression. This case suggests a rarer variant of PRS, and the neurologic findings indicate that the underlying essential neural degeneration may contribute to the processing of pyramidal tract insult or this syndrome.

Unilateral facial contour deformities in two patients with localised lipodystrophies were reconstructed with free radial forearm adipofascial flaps. At follow-up of 5 and 4 years there is no significant downward gravitation, and the flaps have maintained their integrity and bulk. Both the patients have had significant improvement in their self-consciousness of appearance. Since the donor site was closed primarily, a linear forearm scar remains the only donor site morbidity.

Startle-induced epilepsy was observed in a 5-year-old boy with epidermal nevus syndrome. He manifested linear nevus on the face and neck, mild mental retardation, and right hemiparesis. Massive myoclonus, followed by tonic seizures, had been triggered by unexpected auditory stimuli since 3 years of age. The startle-induced seizures were the only epileptic manifestation. Interictal EEG occasionally depicted spontaneous focal spikes and waves in the left frontotemporal area, and ictal EEG depicted vertex spikes and then diffuse slow spike-and-wave complex bursts. Left frontal and perisylvian cortical atrophy and a white matter abnormality in the left frontal area were revealed by magnetic resonance imaging. Single photon emission computed tomography demonstrated diffuse low perfusion in the left cerebral hemisphere. Lower amplitude potentials in the left cerebral cortex were evident during somatosensory evoked potential evaluation. These results indicate that hemispheric dysfunction could cause startle-induced epilepsy in this patient.

We report the case of a 4 year-old boy with Parry-Romberg syndrome who had intractable seizures, progressive cerebral hemisphere atrophy, and fatal brain stem involvement.

Scleroderma rarely presents as total hemiatrophy of one side of the body. A 63-year-old patient is discussed with progressive facial hemiatrophy and with atrophy of skin, subcutaneous tissue, muscle and bone tissue of one side of the body, complicated by ocular and neurological manifestations.

Several operative procedures have been described for correction of soft tissue defects in the face. Free fat transplantation has been criticised many times. In a twelve-year period, we have carried out 19 free fat transfers from the gluteal fold, of a size ranging from the palm of a child's hand to the size of an adult hand. Follow-up has been possible in 12 patients with 14 such transfers. Initially, grafts tended to be too large and subsequent fat reduction was required. It is apparent that the tendency to resorption of non-vascularised fat can only be estimated to a limited extent. Ultrasound examination in the course of long-term follow-up showed structural changes within the grafts but none was particularly striking. The fat has a good consistency on palpation particularly for the cheeks and remains in situ after healing. This technique still has a role in suitable cases.

Four patients who underwent functioning free muscle transplantation (FFMT) for facial reconstruction developed a progressive disfiguring muscle contracture. This complication has not been previously reported. Three of the patients had longstanding facial paralysis and were reanimated by FFMT. The fourth patient had left hemifacial atrophy but without facial paralysis. She also underwent FFMT for augmentation. All four FFMTs were innervated by the ipsilateral facial nerve. Initially, they all had a normal facial appearance at rest during the first few months after FFMT. However, they all developed a progressive severe muscle contracture from 6 to 12 months after FFMT. Continuous spontaneous electrical impulse activity, which stimulated the transferred muscle day and night, may be responsible for the progressive muscle contracture. From the patients' reported sensations and from clinical evaluation, which included a local xylocaine injection test, over-reinnervation with a synkinesis effect of the transferred muscle is hypothetically the main cause, not over-tension of the muscle itself. This complication may possibly be avoided by limiting or decreasing the number of fascicles from the ipsilateral facial nerve or better by using a cross-facial nerve graft instead of the ipsilateral facial nerve as the innervating motor nerve. The outcome with a cross-facial nerve is likely to be more predictable and reliable.

Progressive hemifacial atrophy (Parry-Romberg syndrome) is a slowly progressing facial atrophy of subcutaneous fat and the wasting of associated skin, cartilage, and bone. This disorder includes an active progressive phase (2 to 10 years) followed by a burning out of the atrophic process with subsequent stability. This article presents a review of the literature and a case report with unique dental involvement as a result of this disease process.

Hemifacial atrophy (Parry-Romberg syndrome) is a rare but well-recognized condition. The developmental effects on facial and oral structures have been well documented in the literature. However, the etiology of hemifacial atrophy remains unknown. Previous reported alterations in tooth growth and development include delayed tooth eruption, abnormal root morphology, and malocclusion but not root resorption. This report presents the orofacial changes from hemifacial atrophy in a 22-yr-old woman and describes the occurrence of root resorptions in two regions affected by this condition. The concurrent presence of root resorptions in both the maxilla and mandible in the regions showing the most severe dental malformations points to root resorption as an additional complication of hemifacial atrophy.

Summary We critically review 13 patients with progressive hemifacial atrophy treated with three basic surgical procedures (free flap transplantation, alloplastic implants, micro-fat injections ‘lipofilling’) and further ancillary techniques. In spite of the satisfactory results achieved with the procedures, with the exception of alloplasts, we feel that lipofilling may be considered an interesting solution for soft tissue augmentation of the face especially for moderate adipose defects, due to its repeatability, no donor site morbidity, no complications at the recipient site such as lesions resulting from dissection, bleeding, necrosis, etc. This technique can be performed in a day-hospital with short surgery time, at low cost and without a highly skilled team. For severe grades of adipose atrophy, because of the low blood supply to these tissues which interferes with take of any type of autograft, we think that free flaps actually represent one of the best solutions for soft tissue augmentation.

Pericranial or osteopericranial flaps pedicled on the superficial temporal vessels were used in the correction of various facial deformities in 16 patients. In 5 patients orthognathic surgery was also performed. In all patients a significant aesthetic improvement was achieved. In order to achieve a satisfactory tissue volume and thickness it was necessary to create a folded pericranial flap. It is possible, as a one stage procedure, to combine osteopericranial with pericranial flaps to correct severe facial deformities. It is concluded that by using this method alone or in combination with orthognathic surgery, when indicated, it is possible to achieve good long term aesthetic and functional results.

A case of a newborn with atresia of the transverse colon and right facial hemiaplasia, anophthalmia, and cerebral dysfunction is reported. Colon atresia is a rare cause of congenital bowel obstruction and often associated with other malformations such as abdominal wall defects, gastrointestinal, cardiac, urogenital, and musculosceletal lesions. Facial hemiaplasia may arise in frame of chromosomal defects or as a result of neurovascular compromise caused by congenital amniotic bands. However, the combination of colon atresia and facial hemiaplasia has not been reported before.

Our experience with dermis-fat free flaps and conventional reconstructive procedures for 35 patients with Romberg's disease over a 7-year period is presented. The groin flap was used for increasing volume in 33 cases, the scapular flap in 3 cases, and 2 were treated with the latissimus dorsi free flap. To achieve better contour, secondary procedures such as defatting of the flap, pedicled temporal fascial flaps, cartilage and bone grafts, orthognathic surgery, and other conventional aesthetic procedures were performed in 32 patients. Excellent results were achieved in 9 mildly deformed patients, in 12 out of 16 moderately, and in 3 out of 10 severely affected patients. Four moderate and 5 severe cases were classified as good results. Poor results were achieved in 2 severe cases.

Our objective is to report a rare coexistence of Parry-Romberg disease and ischemic stroke. Here, we report the case of a 34-year-old woman with Parry-Romberg syndrome who developed cerebral infarction. This patient developed sudden left-sided weakness and was admitted to our hospital. Magnetic resonance imaging revealed acute cerebral infarction in the posterior limb of the right internal capsule. The patient had been diagnosed with Parry-Romberg syndrome at the age of 12, and she had a history of migraine without aura. Transesophageal echocardiography revealed a patent foramen ovale, but no atrial septal aneurysm or deep vein thrombosis was observed in the lower extremities. She was treated with 200 mg of aspirin and 10 mg of atorvastatin. Her symptoms gradually improved, and she was discharged 10 days after admission. Parry-Romberg syndrome is a rare disease of progressive hemifacial atrophy with unknown etiology. The potential risk factors for ischemic stroke in Parry-Romberg syndrome include ipsilateral cerebrovascular abnormality or migraine. In addition, patent foramen ovale was identified as a concomitant risk factor in our case.

We describe Adie's pupil and radiologic changes related to Parry-Romberg syndrome in a child who presented with facial hemiatrophy with no neurologic deficit. We suggest that cerebral lesions in Parry-Romberg syndrome without neurologic symptoms must be carefully investigated.

Intractable complex partial seizures developed in a 3-year-old female with normal intracranial findings on computed tomography. Frontal paramedian band-like depression of the skin gradually developed thereafter, and progressive facial hemiatrophy (Parry-Romberg syndrome) was diagnosed. Computed tomography scanning at 5 years of age revealed multiple parenchymal calcifications and low-density areas in the white matter of the frontoparietal lobes. Epileptic seizures, one of the major neurologic complications of progressive facial hemiatrophy, could precede the succeeding neurocutaneous changes.

The anterolateral thigh flap, first described by Song, is one of the septocutaneous flaps in the thigh. Since November 1985 we have transferred 32 anterolateral thigh flaps successfully without any loss. Six of these flaps were performed in Australia. The vascular pedicles are divided into three types: fasciocutaneous perforator (37.5%-12/32), vertical musculocutaneous perforator (56.2%-18/32) and horizontal musculocutaneous perforator (6.6%-2/32). This paper presents the surgical anatomy of the flap and clinical experience.

A radial forearm fascia-fat flap transferred by extracorporeal tissue transfer has been evolved and is presented here for the treatment of Romberg's hemifacial atrophy as an alternative to free tissue transfer. The advantages of this technique are that it transfers a similar kind of tissue which is autogenous, the transferred tissue is not liable to undergo absorption, and it has a natural consistency and less gravitational sag. Donor site morbidity is minimal, it is almost a single stage procedure as the second stage is minor, and most important of all, results similar to free flap transfer can be achieved without the facilities and training required for microsurgery.

A 25-year-old woman is presented with hemifacial atrophy due to unilateral bulbar poliomyelitis infection. Although bulbar poliomyelitis is not an uncommon disease, it is rarely a cause of hemifacial asymmetry.

Four patients with progressive facial hemi-atrophy have been successfully treated with a free vascularised dermis-fat flap. In each case lessons were learnt that led to modifications that proved useful in the next patient. These technical innovations are discussed and a plea is made for surgical treatment of this deformity at an earlier age than is commonly recommended.