Hemiatrophy Research Articles

A rare case of progressive hemifacial atrophy (Parry–Romberg syndrome) — a case report

A rare case of progressive hemifacial atrophy (Parry–Romberg syndrome) — a case report

A Unique Case of Progressive Hemi-facial Atrophy Successfully Treated with Methotrexate

The effects of many dermatologic syndromes are not exclusive to the skin. Disorders commonly involve a complex interplay between multiple organ systems, thus not relying solely on the dermatologist for proper work up, diagnosis, and treatment. Morphea is one such rare disease which involves progressive loss or atrophy of subcutaneous tissue, muscle, and bone with a relatively mysterious etiology. The initial lesion of morphea can be subtle and appear as a pink to red plaque without any additional symptomatology. A biopsy at this early stage is non-specific and will only show the presence of a T cell infiltrate, vascular swelling, and edema. This active or progressive stage will continue for years before “burning out,” or halting progression, although still affecting underlying tissues. Many times, the sclerosis becomes severe enough to cause deformity and secondary systemic symptoms. Five general subtypes of morphea exist, including: plaque-type, linear, deep, guttate, and nodular. In this paper, the authors report a case report of a rare subtype of linear morphea called Parry Romberg syndrome, also known as progressive hemi-facial atrophy (PHA). PHA usually involves at least one branch of the trigeminal nerve unilaterally. The authors will emphasize the importance of a multidisciplinary approach to diagnose and treat this disorder while also considering the multiple theories surrounding its pathophysiology.

Прогрессирующая гемиатрофия лица у ребенка с нарушениями, вызванными мальформацией Арнольда–Киари I типа

Прогрессирующая гемиатрофия лица у ребенка с нарушениями, вызванными мальформацией Арнольда–Киари I типа

Parry-romberg syndrome: about a case.

Parry-romberg syndrome: about a case.

Parry-Romberg Syndrome Associated with Hypothyroidism

Parry-Romberg Syndrome Associated with Hypothyroidism

PARRY ROMBERG SYNDROME

Parry-Romberg syndrome, which is also known as progressive facial hemiatrophy, is a variant of localized scleroderma. This is a rare condition (OMIM # 141300), characterized by a progressive but self-limited atrophy of the skin and subcutaneous tissue on one side of the face. We present the case of a 9 year old boy with a typical presentation of hemifacial atrophy, with Borrelia burgdorferi infection without any changes of the nervous system.

Autologous Fat Grafting in Management of Patients with Progressive Hemifacial Atrophy

Background: Progressive hemi facial atrophy or Romberg disease is a rare neurocutaneous syndrome characterized by slow progressive atrophy on one side of face involving skin, subcutaneous and connective tissue. Various methods have been described for soft tissue augmentation in patients with progressive hemi facial atrophy. Autologous fat, dermofat, fascial grafts have been used for grade 1,2 and 3 atrophy. Free flaps, cartilage and bone grafts are described for grade 3 and 4 atrophy. Aims and objectives: To study efficacy of autologous fat graft in patients with grade 1, 2 and 3 hemi facial atrophy. Strategies to reduce fat absorption and to calculate approximate amount of fat required required by facial mask template. Observations: It is a prospective study conducted in Department of plastic surgery, Bangalore Medical college and Research Institute, Bangalore in 10 patients with grade 2 and 3 Progressive hemi facial atrophy. All patients with stable non-progressive atrophy were graded for severity of disease and face-mask templates were prepared by pro-sthodontists to know approximate amount of fat required for injection. Fat was aspirated by low-pressure small suction cannulas manually and infiltrated at multiple points and in multiple planes after sedimentation and separation of fat. Five of the patients with grade 2 and 3 of patients with grade 3 atrophy were satisfied with symmetric face and with emotional betterment. Two patients needed repeat fat grafting after 2 years of follow-up. Conclusion: Autologous fat grafting is a simple and safe method for augmentation of soft tissue in patients with grade 1, 2 and 3 Progressive hemi facial atrophy. Aspiration of fat with low-pressure small cannulas and infiltrating in multiple planes improves fat survival. Preparing face-mask templates gives an idea of approximate volume of fat required. Keywords: Progressive hemifacial atrophy, Autologous fat grafting, Face-mask templates, Strategies for decreased fat absorption.

Parry-Romberg Syndrome: Report of two cases with review of literature

Parry-Romberg syndrome (PRS) is an uncommon slowly progressing degenerative disease of unknown aetiology, characterized by unilateral atrophic changes on affected part of face involving the skin, fatty tissue, muscles, bone and connective tissue. PRS is a syndrome with diverse complication and associated most common are trigeminal neuralgia, facial paraesthesia, severe headache and epilepsy. Treatment of PRS is a multi-disciplinary approach of various specialities. This article represents such rare cases of PRS in 16 years and 14 years old patients with review of literature. Keywords: En Coup De Sabre, Hemifacial Atrophy, Parry-Romberg Syndrome

Parry-Romberg Syndrome on a Major French Revolution Leader: Mirabeau, 1791.

Parry-Romberg Syndrome on a Major French Revolution Leader: Mirabeau, 1791.

The use of expanded polytetrafluoroethylene in depressed deformities of the face.

Expanded polytetrafluoroethylene (ePTFE) has been extensively used for facial soft tissue augmentation procedures, and is regarded as safe and reliable and suitable as a permanent implant. This implant is generally used in the lower third of the face for lips filling, nasal augmentation, nasolabial folds and chin augmentation, and rarely for congenital or acquired depressed deformities of the face. The aim of the present study was to assess the effects of ePTFE in congenital or acquired depressed deformities of the face. From September, 2008 to January, 2014, 26 patients were implanted with the material ePTFE to correct depressed deformities of the face. The average age at operation was 23.2 years, with a range of 17–45 years. The depressed deformities were lateralized. The follow-up period was 6–18 months (average 9 months). The etiologies of the depressed deformities included stable hemifacial atrophy (3 cases), craniofacial microsomia (13 cases), bony depression after trauma (8 cases), and other unclear reasons (2 cases). The operations were performed under local anesthesia. ePTFE was inserted in different tissue planes that varied among the different subanatomical areas in the face: beneath the superficial temporal fascia in the temporal area, and on the surface of the superficial musculoaponeurotic system in the zygomatic area, cheek and mandibular area. All of the patients were followed up. Most of the patients [25 of 26 patients (96.2%)] were satisfied with the results, while 1 patient (3.8%) was not satisfied for incomplete correction of the depressed deformity. In conclusion, aside from lipofilling and a free flap transfer, the results showed that ePTFE was an alternative treatment for facial depressed deformity.

Autologous Fat Grafting With Combined Three-Dimensional and Mirror-Image Analyses for Progressive Hemifacial Atrophy

Autologous fat grafting (AFG) has been widely used to treat progressive hemifacial atrophy (PHA). However, objective and quantitative analyses in patients with PHA are complicated because of multidimensional atrophy and fat absorption. This study aimed to introduce a new method to predict volumetric deficiency of soft tissues and objectively evaluate the survival of grafted fat with 3-dimensional (3D) and mirror-image analysis (MIA). Thirty-one patients with PHA were classified into 2 groups based on the location of the facial midsagittal plane (FMP), which was established through our proposed method using nasion (point a), anterior nasal spine (point b), and pogonion (point c). The included cases with centered FMP were treated using serial AFG and followed up for 12 to 15 months. Images were obtained preoperatively (pre-OP) and postoperatively at 2 time points (3 months and 12 months). Three-dimensional computed tomography (CT) was performed before the first surgery to locate the FMP and predict the volumetric deficiency of soft tissues with MIA. Three months after the first surgery, 3D laser scanning was conducted to evaluate the volume of surviving fat. Patient characteristics, satisfaction, and soft tissue augmentation results were analyzed. Twenty-seven cases (9 men and 18 women) with PHA were included and treated using serial AFG (mean number of operation, 3.1 times). The mean age was 22.1 years. With 3D CT or 3D laser-scanning camera and MIA, we obtained the mean deficient volume of the affected area (30.48 mm) and the mean volume of the total fat injection (133.61 mm). The mean fat absorption ratio was 47.92%. The mean satisfaction score immediately obtained at first postoperative (score = 4.3) was higher than that at 3 months postoperatively (score = 4.1) and 12 months postoperatively (score = 4.0). No complications and donor-site morbidity were noted. The facial symmetry of patients with centered FMP can be successfully restored using serial AFG alone. The combined 3D and MIA can be used to predict the volumetric deficiency of soft tissues and objectively evaluate the survival of grafted fat.

The second fat graft has significantly better outcome than the first fat graft for Romberg syndrome: A study of three-dimensional volumetric analysis

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. 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. 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<0.01). 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.

Implicaciones anestésicas del síndrome de Parry-Romberg: reporte de un caso

IntroductionParry-Romberg syndrome is a rare degenerative disease characterized by unilateral atrophy affecting the skin, connective tissue, muscle and bone. The end result is facial asymmetry associated with other skin, dental, visual, cardiovascular, and neurological disorders. Clinical findings, diagnostic evaluation and interventionsThe case of a patient with Parry-Romberg syndrome programmed for frontonasal flap remodeling is discussed. The patient's history includes trigeminal neuralgia, epilepsy, and two previous surgical interventions. Uneventful endotracheal intubation with the Glideoscope® video laryngoscope was performed, upon adequate pre-oxygenation followed by anesthetic induction. ConclusionThe phenotypical characteristics of Parry-Romberg syndrome are severe facial hemiatrophy and craniofacial anomalies that require careful preoperative evaluation and management of a potentially difficult airway. Consequently, the use of video laryngoscopes is a first-line approach. Due to the syndrome's associated disorders, it is essential to maintain hemodynamic stability and prevent any potential seizures.

Anesthetic implications of Parry Romberg Syndrome: A case report

IntroductionParry–Romberg Syndrome is a rare degenerative disease characterized by unilateral atrophy affecting the skin, connective tissue, muscle and bone. The end result is facial asymmetry associated with other skin, dental, visual, cardiovascular, and neurological disorders. Clinical findings, diagnostic evaluation and interventionsThe case of a patient with Parry–Romberg Syndrome programmed for frontonasal flap remodeling is discussed. The patient's history includes trigeminal neuralgia, epilepsy, and two previous surgical interventions. Uneventful endotracheal intubation with the Glideoscope® video laryngoscope was performed, upon adequate pre-oxygenation followed by anesthetic induction. ConclusionThe phenotypical characteristics of Parry Romberg Syndrome are severe facial hemiatrophy and craniofacial anomalies that require careful preoperative evaluation and management of a potentially difficult airway. Consequently, the use of video laryngoscopes is a first-line approach. Due to the syndrome's associated disorders, it is essential to maintain hemodynamic stability and prevent any potential seizures.

Late progression of neurological symptoms and MRI T2 hyperintensities in Parry-Romberg syndrome

We describe a case of a 50-year-old woman who developed drug-resistant status epilepticus with complex partial and secondary generalised seizures. She had suffered from Parry-Romberg syndrome (PRS) for more than...

Parry Romberg Syndrome: A Case Report

Parry-Romberg syndrome is a rare disorder characterized by slowly progressive deterioration of the skin and soft tissues of half of the face. The syndrome presents with characteristic skeletal, dental, and soft tissue changes in the affected half of the face, with or without neurological signs and symptoms. The disfiguring nature of the disease Results in psychological disturbance and communication disorders like speech defects, as also dental anomalies. The present article reports a case of a 14-year-old girl who presented with features of Parry Romberg syndrome.

Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease

Parry–Romberg syndrome (PRS) is characterized by progressive degeneration and atrophy of the cutaneous, subcutaneous connective tissues, muscles and bones. Classically, PRS is restricted to unilateral face but in 20% of patients may extend to other parts of the body including ipsilateral or contralateral arms, trunk and legs. We report a case of 24-year-old male who presented with insidious onset, gradually progressive deformity and muscle wasting of right lower limb followed by right side of face and chest for 8 years. The right side of the face showed hemiatrophy, coup de sabre and deviation of nose and chin toward the same side. The magnetic resonance imaging showed atrophy of right lower limb. Computed tomography with 3D facial reconstruction revealed atrophy of facial bones on right side. He was managed with physiotherapy and symptomatic treatment and planned for facial and ankle reconstructive surgery on follow-up.

進行性顔面半側萎縮症患者の上唇変形をCross-lip vermilion flapで再建した1例

進行性顔面半側萎縮症患者の上唇変形をCross-lip vermilion flapで再建した1例

Head injury as a trigger of Parry-Romberg syndrome: A case report and review of etiology

Introduction: this is a case of a patient with the rare entity of Parry-romberg syndrome (Prs) which discuss the etiological association with preceding head trauma as well as the possible therapeutic role of vitamin b12, with a brief review of literature. case report: A 22-year-old female presented with a history of head injury preceding the progressive onset of right-sided facial pain and atrophy, evident facial asymmetry with periorbital hyperpigmentation on the right side of the face, enophthalmos and watering of the right eye. Laboratory investigations revealed a low vitamin b12 level. A final diagnosis of Parryromberg syndrome (“progressive hemifacial atrophy”) was based on clinical and a radiological examination. conclusion: Parry-romberg syndrome is an incompletely understood selflimiting degenerative condition, characterized by unilateral atrophic changes of the face. the pathophysiology of the syndrome remains unclear, but the etiologic relevance of factors like head injury and vitamin b12 deficiency needs to be investigated in further collective studies.

Goldenhar-Gorlin syndrome: A rare case report with review of literature

Goldenhar-Gorlin syndrome (GGS) is a rare developmental anomaly, characterized by numerous anomalies affecting the first and second brachial arches which results in hemifacial atrophy in majority of cases (85%) and associated with cervical spine vertebral deformities, cardiovascular malformation, and sternocleidomastoid muscle abnormalities. Hereby, we report such a rare case of GGS in a 20 year old male patient, with all these classical features.