Progressive hemifacial atrophy, also known as Parry-Romberg syndrome (PRS), is an uncommon condition that causes slow and progressive unilateral soft-tissue atrophy of the face. This atrophy affects the skin and subcutaneous tissue, including fat and muscle, and in some cases, may also involve the underlying bone and muscle. The exact cause of this syndrome is unknown, but several possible factors have been suggested. These include genetic predisposition, autoimmune responses, and infections or trauma. We report a case of a young female who presented with an asymptomatic one-sided atrophy of the face for the last ten years. This case highlights autologous fat grafting as a safe and effective option for restoring facial symmetry in PRS.

Progressive hemifacial atrophy (PHA) is a progressive, irreversible disorder characterized by unilateral facial soft tissue atrophy. Conventional treatments, such as isolated fat grafting or synthetic fillers, are often limited by high resorption rates and insufficient structural support. To address these challenges, a staged surgical protocol integrating autologous fat grafting (AFG) and autologous dermal grafting was implemented to optimize outcomes in moderate-to-severe cases. This study evaluates the synergistic efficacy of combined AFG and dermal grafting in the treatment of PHA. Two patients underwent sequential procedures: initial AFG using the Coleman technique with abdominal fat harvest, followed by dermal grafting from the gluteal region to address residual deep depressions. Postoperative evaluations, including long-term follow-up (up to 20 months), assessed volumetric retention, complications, and patient satisfaction. Results demonstrated significant improvements in facial symmetry and contour, with dermal grafts providing durable structural reinforcement in areas where fat resorption persisted. No major complications were observed, and both patients reported high satisfaction with aesthetic outcomes. The staged integration of AFG and dermal grafting offers a minimally invasive, biocompatible solution for complex PHA reconstruction, leveraging dynamic adaptability and natural tissue integration. This dual-modality approach presents a promising strategy for recalcitrant soft tissue defects, particularly where traditional fat grafting alone proves insufficient.

Parry Romberg Syndrome (PRS) is a less common genetic condition presenting with progressive hemifacial atrophy involving skin, underlying connective tissue, muscle and facial bone. Neurological manifestations include seizures, headaches, deafness and trigeminal neuralgia refractory to medications, while stroke is a less common presentation. A 43-year-old right-handed female with previous history of Lower Motor Neuron (LMN) type facial palsy, seizure disorder and linear scleroderma, presented to our clinic with recurrent cryptogenic strokes. She developed progressive hemifacial atrophy on the left side and left eye ectropion and was eventually diagnosed with rare Parry Romberg Syndrome. Patient underwent extensive work up for stroke to rule out etiologies like hyperlipidemia, diabetes, lupus and vasculitis. Peripheral labs for inflammatory markers and Cerebrospinal fluid (CSF) studies were unremarkable. Brain imaging at different points in time showed progressive atrophy of brain parenchyma, overlying bone, connective tissue and facial muscles on the left side. Central Nervous System (CNS) vessel imaging and diagnostic cerebral angiogram was unremarkable. This novel case underscores the potential CNS involvement in PRS, which is a rare disease entity. Neurological manifestations are not uncommon, including stroke. Further research is needed to understand the mechanisms of stroke in this rare disease process, that could help develop potential therapeutic targets.

Progressive hemifacial atrophy, also known as Parry-Romberg syndrome (PRS), is an idiopathic disorder characterized by progressive unilateral facial depression caused by atrophy of the skin and subcutaneous tissue. We present a case of mandibular osteomyelitis occurring in a patient with this condition, together with a review of the literature. The clinical findings in this case suggest that autoimmune disease and trauma are involved in contributing factors to disease progression. Possible involvement of neurovascular and neuroskeletal dysregulations in the pathogenesis of PRS is also discussed.

Parry-Romberg syndrome (PRS), also known as progressive facial hemiatrophy, is a rare neurocutaneous disorder characterized by progressive, unilateral atrophy of the facial tissues. It usually appears during childhood or early adulthood; however, adult-onset cases are increasingly being recognized. PRS significantly overlaps with localized scleroderma, especially the en coup de sabre (ECDS) subtype, indicating a common pathophysiological mechanism. We present a 52-year-old female patient with adult-onset PRS associated with ECDS. The patient was first diagnosed with localized scleroderma in 1999, when she developed subtle atrophic changes in the right side of her face. Over two decades, the atrophy gradually advanced, leading to marked facial asymmetry. Re-evaluation in 2023 showed right hemifacial atrophy with a linear "sword stroke" lesion over the forehead that is a characteristic of ECDS. Laboratory tests, imaging, ophthalmologic evaluation, and skin biopsies confirmed PRS with linear scleroderma. To address the progressive functional and aesthetic impairments, systemic treatment with methotrexate was initiated to halt disease progression, with regular follow-ups scheduled for monitoring. This rare case of adult-onset PRS evolving from localized scleroderma highlights the need for high clinical suspicion in adults with progressive hemifacial atrophy, especially with ECDS. Early diagnosis and timely immunosuppressive treatment are crucial to limit disease progression and maximize patient outcomes. Enophthalmos is defined as the posterior displacement of the globe within the anteroposterior plane of the orbit, producing the clinical impression of a sunken eye (see Image. Orbit, Anterior View). The condition represents a descriptive clinical finding rather than a diagnosis, indicating an underlying imbalance among orbital structures. Under normal conditions, the globe occupies a stable position within the orbital cavity, maintained by equilibrium among the bony orbital walls, orbital fat, extraocular muscles, connective tissue septae, and vascular components. Disruption of this anatomical harmony—whether from trauma, inflammation, infection, congenital anomalies, or degenerative processes—can result in globe displacement. Recognition of enophthalmos provides a significant clinical clue to local and systemic pathologies affecting the orbit. Differentiation from pseudo-enophthalmos, which reflects an apparent posterior position of the globe due to structural asymmetry or ocular abnormalities, is essential. Common causes of pseudo-enophthalmos include microphthalmos, phthisis bulbi, and anophthalmic socket, in which a small or absent globe produces a deceptive impression of posterior displacement. Globe malpositions, such as hyperglobus, hypoglobus, esoglobus, or exoglobus, as well as contralateral proptosis, can also simulate enophthalmos. Accurate distinction between true and pseudo-enophthalmos is critical for appropriate diagnosis and management. Clinically, enophthalmos manifests as a posteriorly displaced globe, often accompanied by a deep superior sulcus (superior sulcus deformity) and a hollowed periorbital contour. Eyelid position may be variably affected, including ptosis, retraction, or lagophthalmos, and severe cases can result in functional disturbances such as diplopia, restricted ocular motility, or impaired eyelid closure. The opposite clinical finding is proptosis (exophthalmos), defined by anterior displacement of the globe. While proptosis is often more overt due to its cosmetic prominence, enophthalmos typically develops gradually and may remain unnoticed until functional or aesthetic complications arise. From an anatomical perspective, the orbit is a pyramidal cavity formed by the frontal, zygomatic, maxillary, ethmoid, lacrimal, sphenoid, and palatine bones. The orbital cavity contains the globe, extraocular muscles, adipose tissue, neurovascular structures, and fascial septae, which collectively cushion and stabilize the eyeball. Approximately 40% of the orbital volume consists of fat, a critical determinant of globe position. The integrity of the orbital walls and the volume of soft tissue together define ocular projection. The pathophysiology of enophthalmos can be classified into 3 principal mechanisms: loss or atrophy of orbital fat, structural expansion of the bony orbit, and fibrosis or scarring of orbital soft tissues that retract the globe posteriorly. Posttraumatic enophthalmos is one of the most common acquired forms, typically resulting from blowout fractures of the orbital floor or medial wall. Herniation of orbital fat into adjacent sinuses increases the effective orbital volume, displacing the globe posteriorly. Chronic maxillary sinusitis, also referred to as “silent sinus syndrome,” can similarly produce progressive inward bowing of the orbital floor, resulting in spontaneous enophthalmos in the absence of overt trauma. Inflammatory and infectious processes can also alter orbital anatomy. Chronic granulomatous disease, orbital pseudotumor, and postinflammatory fibrosis may lead to contracture of periorbital tissues and scarring, tethering the globe. Iatrogenic causes include radiotherapy-induced fat atrophy and fibrosis, often observed after orbital irradiation for malignancies such as retinoblastoma or nasopharyngeal carcinoma. Progressive hemifacial atrophy (Parry-Romberg syndrome) and scleroderma can produce localized orbital tissue loss, contributing to unilateral enophthalmos. Congenital and developmental anomalies also contribute to enophthalmos. Microphthalmos and craniofacial malformations, such as Crouzon or Apert syndromes, may result in asymmetric globe positioning. In these cases, the apparent enophthalmos arises from dysmorphic orbital bones or reduced globe size rather than true posterior displacement. Postsurgical and postenucleation socket contracture can similarly simulate enophthalmos due to inadequate prosthetic support or soft tissue scarring. The natural history of enophthalmos is largely determined by its underlying etiology. Traumatic cases may become apparent weeks to months after the initial injury, as edema resolves and tissue remodeling occurs. Inflammatory or degenerative causes typically progress gradually, with slow orbital fat loss or fibrosis resulting in increasing asymmetry. Early recognition and intervention are critical, as chronic enophthalmos can lead to secondary changes, including fibrosis of the extraocular muscles, restrictive strabismus, and orbital volume distortion, which complicate reconstructive efforts. Clinical assessment requires careful inspection of facial symmetry in multiple gaze positions and measurement using exophthalmometry. A difference greater than 2 mm between both eyes is generally considered significant. Palpation of orbital rims may reveal step deformities in posttraumatic cases, while ocular motility assessment evaluates extraocular muscle involvement. Imaging, particularly high-resolution computed tomography (CT), provides detailed information regarding orbital wall integrity, fat distribution, and muscle morphology. Magnetic resonance imaging (MRI) offers superior soft-tissue resolution, facilitating differentiation between fat atrophy, fibrosis, and infiltrative lesions. A detailed understanding of orbital anatomy is essential for correlating clinical findings with radiologic evidence. The inferior orbital wall, thin and adjacent to the maxillary sinus, is the most common site of trauma-induced defects. The medial wall, composed of the lamina papyracea of the ethmoid bone, is also susceptible. Expanding these walls increases the volume of the orbital cavity, and even minor volumetric changes can substantially alter the globe's position due to the confined orbital space. Conversely, reduction of retrobulbar soft tissue volume, particularly adipose tissue, produces posterior displacement of the globe without skeletal alteration. Orbital pathology influencing enophthalmos varies widely. Inflammatory and infectious processes may extend from adjacent sinuses, particularly the maxillary and ethmoidal sinuses, causing osteolysis and remodeling of the orbital walls. Malignant lesions, such as metastatic carcinoma or orbital lymphoma, may induce bone resorption and fibrosis, indirectly contributing to enophthalmos. Infiltrative processes that primarily expand orbital tissues, such as thyroid-associated orbitopathy, typically cause proptosis but may also produce secondary enophthalmos during the fibrotic phase. Enophthalmos has substantial functional and aesthetic consequences. The sunken appearance of the eye, combined with asymmetrical eyelid contour, can lead to psychological distress and self-consciousness. Functionally, altered globe position impairs binocular vision, restricts ocular motility, and disrupts tear film dynamics, predisposing to exposure keratopathy. Lagophthalmos and incomplete lid closure exacerbate ocular surface compromise, particularly when associated with facial nerve dysfunction or cicatricial lid changes.