OBJECTIVE: To present a case of progressive hemifacial atrophy in a young woman with Parry-Romberg Syndrome and the role of autologous fat transfer to improve her aesthetic appearance and lessen facial asymmetry.

METHODS:

Design:           Case Report

Setting:           Tertiary Government Training Hospital

Patient:           One

RESULTS: A 20-year old woman consulted because of drooping of the right eyelid and gradual thinning of right cheek muscles since age 16.  On examination, the right facial muscles were hypoplastic with prominent facial bony ridges. An MRI scan showed atrophy of the right medial pterygoid and masseter. She underwent autologous fat transfer on the right side of the face to augment the cheek, improve cosmetic appearance and lessen facial asymmetry.  

Conclusion: Our patient is satisfied and happy with the outcome and cosmetic appearance of her autologous fat transfer and is ready to undergo the same procedure if the need arises in the future. Although no definite cure exists for Parry Romberg syndrome, our report illustrates the role of autologous fat transfer as an inexpensive, easily harvested and biocompatible material to improve facial asymmetry. The procedure yielded encouraging results, although long-term benefits remain uncertain.

 

Keywords: Parry-Romberg syndrome; progressive hemifacial atrophy; autologous transplantation

Human ; Female ; Adult (a Person 19-44 Years Of Age) ; Facial Hemiatrophy ; Transplantation, Autologous

Parry-Romberg syndrome is a rare neurocutaneous syndrome characterized by progressive shrinkage and degeneration of the tissues usually on only one side of the face. It is usually difficult to restore the facial contour due to skin tightness. In this case report, we report a forehead reconstruction with custom-made three-dimensional (3D) titanium implant of a Parry-Romberg syndrome patient who was treated with multiple fat grafts but had limited effect. A 36-year-old man presented with hemifacial atrophy. The disease progressed from 5 to 16 years old. The patient had alopecia on frontal scalp and received a surgery using tissue expander. The alopecia lesion was covered by expanded scalp flap done 22 years ago. Also, he was treated with fat grafts on depressed forehead 17 years ago. However, it did not work sufficiently, and there was noted depressed forehead. We planned to make 3D titanium implant to cover the depressed area (from the superior orbital rim to the vertex). During the operation, we confirmed that the custom-made 3D implant accurately fit for the depressed area without any dead spaces. Previously depressed forehead and glabella were elevated, and the forehead contour was improved cosmetically. A custom-made 3D titanium implant is widely used for skull reconstruction and bring good results. In our case, the depressed forehead of a Parry-Romberg syndrome patient was improved by a 3D titanium implant.
Adult ; Alopecia ; Facial Hemiatrophy* ; Forehead* ; Humans ; Neurocutaneous Syndromes ; Orbit ; Scalp ; Skin ; Skull ; Tissue Expansion Devices ; Titanium* ; Transplants

Parry Romberg Syndrome (PRS), also known as idiopathic progressive hemifacial atrophy, is a rare neurocutaneous disorder characterized by loss of skin and subcutaneous fat of face, muscles, and bones causing unilateral atrophy. Most patients require only soft tissue augmentation although syndrome has varying grades of severity. In the majority of reported cases, it has been treated with surgical flap or autologous fat transplantation. However, these treatments need complicated surgical skills which take a lot of time and cost. Herein we report the first case of PRS augmented by hyaluronic acid (HA) filler in a 42-year-old female patient to suggest that HA filler could be a safe, simple, and even rational economic alternative to surgical treatment.
Adult ; Atrophy ; Facial Hemiatrophy* ; Female ; Humans ; Hyaluronic Acid* ; Muscles ; Neurocutaneous Syndromes ; Skin ; Subcutaneous Fat ; Surgical Flaps

PURPOSE: We report a case of Parry-Romberg syndrome associated with right enophthalmos, hypotropia, up-gaze limitation, en coup the sabre, morphea, and hypothyroidism. CASE SUMMARY: A 41-year-old woman presented with slowly progressive right enophthalmos that started 5 years previous. With a Hertel exophthalmometer, the right eye measured 10 mm, and the left eye 13 mm. Right enophthalmos and atrophy of subcutaneous tissues of right side orbit were observed. Axial scan of computerized tomography shows atrophy of right side orbital subcutaneous tissues without bony abnormality. At primary gaze, 4 prism diopter hypotropia was observed in the right eye. Up-gaze was limited in both eyes. Visual acuity was 1.0 in both eyes. No specific sign was found in her anterior segment. The patient had experienced right side alopecia 20 years prior and had undergone biopsy, which proved morphea. Linear en coup de sabre morphea was found on the right forehead and scalp. Hypothyroidism due to Hashimoto's thyroiditis was diagnosed based on a blood test, which also showed positive antinuclear antibody. Thus, the patient was diagnosed with Parry-Romberg syndrome associated with hypothyroidism. CONCLUSIONS: Clinicians need to consider the rare disease Parry-Romberg syndrome when a patient with ophthalmologic symptoms such as enopthlamos or eye movement disorders is accompanied by dermatologic symptoms such as linear en coup de sabre morphea or alopecia.
Adult ; Alopecia ; Antibodies, Antinuclear ; Atrophy ; Biopsy ; Enophthalmos ; Facial Hemiatrophy* ; Female ; Forehead ; Hematologic Tests ; Humans ; Hypothyroidism* ; Ocular Motility Disorders ; Orbit ; Rare Diseases ; Scalp ; Scleroderma, Localized ; Subcutaneous Tissue ; Thyroid Gland ; Thyroiditis ; Visual Acuity

A 19-year-old male patient presented with facial hemi-atrophy with unilateral spasms of the masseter and temporalis muscles. Ultrasound therapy and Transcutaneous Electric Nerve Stimulation therapy, known as combination therapy, were given on alternate days for 2 weeks. At the end of 2 weeks of combination therapy the patient reported a drastic reduction in the number of episodes of muscle spasm. The visual analog scale score for tenderness of the masseter and temporalis was also markedly reduced. No one has previously used combination therapy for the treatment of facial hemi-atrophy with hemi-facial spasms. The encouraging results of the combination therapy has prompted us to document this study.
Botulinum Toxins ; Facial Hemiatrophy* ; Hemifacial Spasm* ; Humans ; Male ; Masseter Muscle ; Muscles ; Spasm ; Transcutaneous Electric Nerve Stimulation ; Ultrasonography ; Visual Analog Scale ; Young Adult

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied. Brain magnetic resonance imaging revealed hemiatrophy of the face and skull base, and T2-weighted images showed increased signal in the right hemipons and hemicerebellar peduncle. Magnetic resonance angiography findings were unremarkable. She was treated with oral prednisolone, and her recurrent gait ataxia diminished within 2 months of the follow-up period. To the best of our knowledge, this is only the second case of PRS presenting with an abnormal involvement of the ipsilateral hemipons.
Ataxia* ; Atrophy ; Brain ; Brain Stem ; Cheek ; Child ; Facial Hemiatrophy* ; Female ; Follow-Up Studies ; Gait Ataxia ; Humans ; Leg ; Magnetic Resonance Angiography ; Magnetic Resonance Imaging ; Muscles ; Prednisolone ; Rabeprazole ; Scalp ; Scleroderma, Localized ; Skin ; Skull Base

Disease Progression ; Facial Hemiatrophy ; surgery ; Humans

OBJECTIVETo sum up the various procedures for skeleton and soft tissue contour reconstruction in severe progressive hemifacial atrophy.

METHODSFrom Jan 2004 to May 2012, 25 patients with severe progressive hemifacial atrophy underwent the procedures of lipoinjection, microsurgical flap transplantation, dermis grafting, distraction osteogenesis, orthognathic surgery and so on for both skeleton and soft tissue reconstruction.

RESULTSAmong them, zygomatic augmentation and lipoinjection were performed in 24 cases, anterolateral thigh adipofascial flap in 10 cases and latissimus dorsi flap in one case, orthognathic surgery in 17 cases, including Le Fort I osetoectomy in 3 cases, genioplasty in 4 cases, mandibular distraction osteogenesis combined with secondary Le Fort I osteotomy in 3 cases, genioplasty combined with mandibular augmentation with Medpor implant in 7 cases. The patients were followed up for 6 months to 5 years. Through skelton and soft tissue reconstruction, the oblique occlusion plane and malocclusion were corrected with great improvement in face asymmetry.

CONCLUSIONSFor severe progreassive hemifacial atrophy, comprehensive procedures should be adopted for both skelton and soft tissue reconstruction to achieve good results.

Adipose Tissue ; transplantation ; Facial Asymmetry ; surgery ; Facial Hemiatrophy ; surgery ; Humans ; Mandible ; surgery ; Orthognathic Surgical Procedures ; Osteogenesis, Distraction ; methods ; Osteotomy, Le Fort ; methods ; Reconstructive Surgical Procedures ; methods ; Skin Transplantation ; methods ; Surgical Flaps ; transplantation

Progressive facial hemiatrophy, also known as Parry-Romberg syndrome, is a progressive and self-limited deformation of the subcutaneous tissue volume on one side of the face that creates craniofacial asymmetry. We present the case of a patient with a five-year history of progressive right facial hemiatrophy, who underwent facial volumetric restoration using cell-assisted lipotransfer (CAL), which consists of an autologous fat graft enriched with adipose-derived stem cells (ASCs) extracted from the same patient. ASCs have the capacity to differentiate into adipocytes. They also promote angiogenesis, release angiogenic growth factors, and some can survive as stem cells. The use of autologous fat as a filler in soft tissue atrophy has been satisfactory in patients with mild and moderate Parry-Romberg syndrome. Currently, CAL has showed promising results in the long term by decreasing the rate of fat reabsorption. The permanence and stability of the graft in all the injected areas has showed that autologous fat grafts enriched with stem cells could be a promising technique for the correction of defects caused by this syndrome.
Adipocytes ; Atrophy ; Facial Hemiatrophy ; Humans ; Intercellular Signaling Peptides and Proteins ; Stem Cells ; Subcutaneous Tissue ; Tissue Therapy ; Transplants

Parry-Romberg syndrome, or progressive facial hemiatrophy, is a rare and acquired neurocutaneous disease of unknown etiology that is classically characterized by progressive and self=limited atrophy of the skin, subcutaneous tissues, and underlying bone structures. Ophthalmic involvement is reportedly not uncommon, and the most common ocular finding is enophthalmos. Here we report a 46=year=old woman with Parry=Romberg syndrome who presented with intermittent exotropia of the right eye. To the best of our knowledge, this is the first description of intermittent exotropia in a patient with Parry-Romberg syndrome.
Atrophy ; Enophthalmos ; Exotropia ; Eye ; Facial Hemiatrophy ; Female ; Humans ; Skin ; Subcutaneous Tissue