Reza Motalebi

and 4 more

IntroductionJuvenile localized scleroderma (JLS) encompasses a group of rare disorders that primarily affect the skin and subcutaneous tissues, with rare involvement of internal organs. The common classification includes five subtypes: circumscribed morphea, linear scleroderma, generalized morphea, pansclerotic morphea, and mixed subtype(1). The incidence in children is 3.4 cases per million children per year, with the vast majority of cases comprising the linear subtype. Linear scleroderma ”en coup de sabre” (LSc-ECDS) is a localized subtype of morphea that is specifically confined to the frontoparietal region of the head and is characterized by a linear, atrophic, and indurated plaque on the forehead and scalp(2).The etiology of LSc-ECDS remains unclear, but current treatments are largely based on low levels of evidence, such as case reports and case series, along with expert opinions. Untreated progression can lead to facial hemiatrophy, joint involvement (in up to 19% of cases, particularly in the linear subtype), and neurologic complications such as seizures, headaches, behavioral changes, or learning disabilities, which occur in approximately 20% of patients with extracutaneous involvement(3).Management of JLS, particularly LSc-ECDS, depends on the subtype, lesion location, and degree of disease activity. Systemic corticosteroids combined with methotrexate (MTX) are reported as effective first-line therapy for active disease, especially in progressive linear scleroderma; MTX at a weekly dose of 15 mg per square meter, along with corticosteroids as a bridge therapy, has demonstrated a 100% response rate in studies(4). For inactive lesions, reconstructive surgical options such as excision and reconstructive techniques are used to improve aesthetic aspects. In refractory cases, mycophenolate mofetil or biologics (such as TNF or IL-6 inhibitors) may be considered, although evidence is limited. Due to the rarity of the disease, referral to specialized pediatric rheumatology centers for evaluation and treatment is recommended(5).This case report describes a rare congenital case of LSc-ECDS in a 7-year-old girl with a family history of systemic lupus erythematosus, which was managed with surgical excision and Z-plasty reconstruction without complications. The uniqueness of this case lies in its congenital onset, absence of systemic or neurological involvement, and the success of early surgical intervention, contributing to the medical literature by emphasizing the role of surgery in atypical pediatric cases to achieve optimal aesthetic and functional outcomes.

Hasan Momeni

and 3 more

IntroductionBimaxillary orthognathic surgery (BOMS) serves as a standard approach for correcting severe skeletal-dental discrepancies, such as Class III malocclusion, significantly enhancing masticatory function, facial aesthetics, and patients’ quality of life(1).However, arch space deficiency affects 30-40% of BOMS candidates, complicating preoperative planning, particularly when impacted wisdom teeth are positioned in the mandibular lesser segment, necessitating extraction to ensure postoperative fixation stability(2, 3). The prevalence of impacted third molar impaction in the general population is approximately 27%, but in orthognathic patients with arch space deficiency, this rate exceeds 50%, potentially leading to complications such as root displacement, bone volume loss, and occlusal instability(3, 4).Recent advancements in virtual surgical planning (VSP) and 3D-printed surgical guides have increased intraoperative accuracy by up to 25% and reduced operative time, which is crucial for managing complex cases like extraction from the lesser segment(5, 6).Recent studies demonstrate the superiority of VSP over traditional methods in transferring planning to the intraoperative phase, with mean errors below 1 mm in three-dimensional displacements(7).Despite these benefits, case reports on the application of 3D guides in combining severe arch space deficiency and third molar extraction during BOMS remain limited to fewer than 5 in the recent literature, highlighting a significant knowledge gap(8).This case report describes the innovative application of a 3D-printed surgical guide for safe and minimally invasive extraction of a third molar from the lesser segment during BOMS in a 19-year-old female patient. By emphasizing bone volume preservation and enhanced surgical precision, this case not only addresses rare structural challenges but also provides a practical and educational model for surgeons, justifying its publication for advancing clinical standards in oral and maxillofacial surgery.

Mehdi Abrishami

and 3 more

IntroductionNeurofibromatosis type 1 (NF1) is one of the most common autosomal dominant genetic disorders, with an estimated prevalence of 1 in 2,500 to 3,000 individuals worldwide. It results from pathogenic variants in the NF1 gene on chromosome 17, leading to haploinsufficiency and dysregulation of the RAS/MAPK signaling pathway, which manifests as a multisystem condition with variable expressivity(1). Characteristic features include café-au-lait macules, axillary freckling, cutaneous neurofibromas, Lisch nodules, optic pathway gliomas, and skeletal abnormalities, with nearly half of cases arising de novo(2). Oral manifestations in NF1, such as gingival hyperplasia, mandibular enlargement, and increased risk of periodontitis or salivary gland dysfunction, become more prominent with age and may contribute to reduced quality of life, though they are often underrecognized in routine dental care(3).Oral lichen planus (OLP) is a chronic T-cell-mediated inflammatory mucocutaneous disorder affecting approximately 0.5-2% of the general population, predominantly middle-aged women, with a potential for malignant transformation to oral squamous cell carcinoma in 1-2% of cases(4). Clinically, it presents as bilateral white reticular striae, erosions, or plaques on the buccal mucosa, often accompanied by symptoms of discomfort or burning sensation, and histopathologically characterized by basal cell degeneration, band-like lymphocytic infiltrate, and Civatte bodies(5). While autoimmune and environmental triggers are implicated, the precise pathogenesis involves dysregulated immune responses, including exosome-mediated cytokine release (e.g., IL-6, TNF-α), highlighting the need for non-invasive biomarkers for early detection(6).To date, direct associations between OLP and NF1 remain exceedingly rare, with only isolated reports describing verruciform xanthoma in NF1 patients exhibiting clinical features mimicking OLP, suggesting possible overlapping inflammatory pathways influenced by NF1-related fibrosis(7). This case report aims to describe the clinical, histopathological, and genetic features of biopsy-confirmed erosive OLP in a 22-year-old woman with familial NF1 harboring a pathogenic NF1 variant (c.2850+1delG), representing a novel documentation of this comorbidity and underscoring the importance of multidisciplinary surveillance in NF1 patients.

Saleh Mohebbi

and 3 more

IntroductionBony sequestration, also known as sequestrum formation, is a well-documented pathological process characterized by the separation of necrotic bone from viable surrounding tissue, typically observed in the context of osteomyelitis or malignancy. In the maxillofacial region, this condition predominantly affects the mandible due to its relatively limited vascular supply; however, maxillary involvement, though less frequent, presents unique clinical challenges [1].The maxilla’s rich vascularity and porous membranous structure typically confer resistance to osteomyelitis, with a mandible-to-maxilla involvement ratio of approximately 3:1[2]. Predisposing factors for jawbone osteomyelitis include trauma, radiotherapy, and pharmacotherapies such as bisphosphonates and denosumab, which disrupt bone remodeling and increase susceptibility to necrosis [3, 4]. Radiation-induced osteonecrosis, a recognized complication of head and neck cancer treatment, further exacerbates this risk by impairing vascular integrity and promoting hypoxic tissue damage [5].The emergence of mucormycosis as a significant etiological factor in maxillary osteomyelitis has gained attention, particularly in the post-COVID-19 era. Mucormycosis, caused primarily by Mucorales species, is an angioinvasive fungal infection that thrives in immunocompromised states, infiltrating blood vessels and inducing thrombosis and necrosis [6]. Its predilection for the maxilla is attributed to anatomical proximity to the paranasal sinuses, a common entry point for fungal spores [7] .Pre-COVID-19 literature reported maxillary fungal osteomyelitis as a rare entity, with incidence rates favoring the maxilla (52%) over the mandible and a male-to-female ratio of 2.1:1 [8]. However, since 2020, a dramatic rise in mucormycosis cases has been documented among COVID-19 patients, particularly in India, linked to corticosteroid use, hyperglycemia, and immune dysregulation [9]. Pal et al. (2021) reported that Mucorales species accounted for 44% of fungal osteomyelitis cases, with Aspergillus spp. contributing a smaller fraction (2%) [10].Clinically, maxillary bony sequestration presents with pain, swelling, sinus tracts, and occasionally exposed necrotic bone, often mimicking other odontogenic or neoplastic conditions [11]. Radiographic evaluation remains a cornerstone of diagnosis. Plain radiographs and computed tomography (CT) reveal sequestra as calcified fragments within lucent lesions, with multiplanar CT reconstructions enhancing detection sensitivity [12]. However, these modalities struggle to differentiate necrotic bone from residual viable bone or calcified matrix [13]. Magnetic resonance imaging (MRI) offers superior soft-tissue contrast, distinguishing avascular sequestra within necrotic zones from vascularized bone in viable tissue when enhanced with gadolinium [14]. Nevertheless, MRI’s limitations in detecting small calcifications underscore the need for combined CT-MRI approaches [15].The post-COVID-19 surge in mucormycosis-associated osteomyelitis has introduced new diagnostic and therapeutic challenges. Studies indicate that COVID-19 exacerbates susceptibility to opportunistic infections through systemic inflammation and immune suppression, with fungal osteomyelitis emerging as a life-threatening complication (Mehta & Pandey, 2020). Treatment typically involves surgical debridement of necrotic tissue, antifungal therapy (e.g., amphotericin B), and management of underlying predispositions, though outcomes vary widely based on disease extent and patient comorbidities (Skiada et al., 2018).Despite advances in understanding maxillary bony sequestration and its association with mucormycosis in the post-COVID-19 context, several knowledge gaps persist. First, the precise mechanisms linking COVID-19-induced immune dysregulation to enhanced fungal pathogenicity remain poorly elucidated, limiting targeted preventive strategies. Second, the rarity of maxillary involvement prior to the pandemic complicates establishing baseline incidence rates and risk profiles specific to this site, particularly in immunocompetent populations. Third, while imaging modalities like CT and MRI have improved diagnostic precision, their ability to predict sequestrum viability and guide surgical planning remains suboptimal, necessitating novel imaging or biomarker-based approaches. Finally, the long-term outcomes of mucormycosis-associated osteomyelitis in COVID-19 survivors, including recurrence rates and quality of life, are inadequately documented, hindering the development of evidence-based management protocols. Addressing these gaps is critical to improving clinical outcomes in this emerging and complex disease entity.This case report explores the etiology, clinical presentation, and management of post-COVID-19 maxillary bony sequestration, highlighting its association with mucormycosis and the diagnostic challenges posed by this emerging complication.