Anesthetic management of a patient with mandibular hypoplasia, deafness, progeroid features, lipodystrophy syndrome: a case report.

BACKGROUND: Mandibular hypoplasia, deafness, progeroid features, and lipodystrophy (MDPL) syndrome is a rare autosomal dominant disorder that presents unique challenges for anesthetic management due to its multisystemic manifestations. This report outlines the anesthetic considerations for MDPL patients based on our case experience. CASE PRESENTATION: A 15-year-old male with MDPL syndrome underwent testicular extraction under general anesthesia. Insertion of a peripheral venous catheter was challenging due to scleroderma-like skin. Although the facial features of MDPL syndrome suggested a difficult airway, intubation with a McGrath™ Mac video laryngoscope was successful. Despite MDPL syndrome's association with hypertriglyceridemia due to lipodystrophy, this patient's triglyceride levels were normal. Thiamylal and sevoflurane were used without issues such as delayed emergence from anesthesia. CONCLUSIONS: MDPL syndrome requires careful preoperative assessment and tailored anesthetic management due to potential airway challenges arising from its distinctive facial features and the possibility of altered anesthetic pharmacokinetics associated with lipodystrophy.

Complexities of Hemifacial Microsomia: A Case Study of Mandibular Hypoplasia and Ear Deformity.

Hemifacial microsomia (HFM) presents a complex congenital anomaly characterized by the asymmetric underdevelopment of facial structures, predominantly affecting the ear, mouth, and mandible on one side of the face. This case study examines the intricacies of HFM through the presentation of a 23-year-old female with congenital deformities of the left ear and mandibular hypoplasia. Clinical and radiological evaluations revealed significant facial malformations, including mandibular hypoplasia, left temporomandibular joint fusion, and maxillary abnormalities, confirming the diagnosis of HFM. Management of HFM necessitates a multidisciplinary approach involving otolaryngologists, maxillofacial surgeons, orthodontists, and audiologists to comprehensively address functional and aesthetic concerns. Early diagnosis and intervention, along with psychosocial support, are essential for optimizing outcomes and improving the quality of life for individuals with HFM.

Case report: A novel splice-site mutation of MTX2 gene caused mandibuloacral dysplasia progeroid syndrome: the first report from China and literature review.

Background: Mandibuloacral dysplasia (MAD) syndrome is a rare genetic disease. Several progeroid syndromes including mandibuloacral dysplasia type A (MADA), mandibuloacral dysplasia type B(MADB), Hutchinson-Gilford progeria (HGPS) and mandibular hypoplasia, deafness, and lipodystrophy syndrome (MDPL) have been reported previously. A novel MAD progeroid syndrome (MADaM) has recently been reported. So far, 7 cases of MADaM diagnosed with molecular diagnostics have been reported in worldwide. In the Chinese population, cases of MAD associated with the MTX2 variant have never been reported. Methods: The clinical symptoms and the genetic analysis were identified and investigated in patients presented with the disease. In addition, we analyzed and compared 7 MADaM cases reported worldwide and summarized the progeroid syndromes reported in the Chinese population to date. Results: The present study reports a case of a novel homozygous mutation c.378 + 1G > A in the MTX2 gene, which has not been previously reported in the literature. Patients present with early onset and severe symptoms and soon after birth are found to have growth retardation. In addition to the progeroid features, skeletal deformities, generalized lipodystrophy reported previously, and other multisystem involvement, e.g. hepatosplenic, renal, and cardiovascular system, this case was also reported to have combined hypogammaglobulinemia. She has since been admitted to the hospital several times for infections. Among 22 previously reported progeroid syndromes, 16/22 were MADA or HGPS caused by LMNA gene mutations, and the homozygous c.1579C > T (p.R527C) mutation may be a hot spot mutation for MAD in the Chinese population. MAD and HGPS mostly present in infancy with skin abnormalities or alopecia, MDPL mostly presents in school age with growth retardation as the first manifestation, and is often combined with an endocrine metabolism disorder after several decades. Conclusion: This is the first case of MAD syndrome caused by mutations in MTX2 gene reported in the Chinese population. MTX2 gene c.378 + 1G > A homozygous mutation has not been previously reported and the report of this patient expands the spectrum of MTX2 mutations. In addition, we summarized the genotypes and clinical characteristics of patients with progeroid syndromes in China.

Goldenhar syndrome with limbal neoformation, microtia and skeletal deformities: a case report and literature review.

BACKGROUND: To report a case of a 4-year-old patient with Goldenhar syndrome. CASE PRESENTATION: The author presents a rare case report involving a 4-year-old boy with multiple malformations. A comprehensive examination showed that the patient primarily had a limbal dermoid. He also has bilateral microtia and ear canal deformities. The skull CT scan and spine X-ray showed Maxillofacial Abnormalities and scoliosis. Whole Exome Sequencing revealed potential gene variations related to microtia. Although certain circumstances prevented us from initiating follow-up treatment for the patient, we have provided a detailed account of the diagnostic methodologies used for this condition. CONCLUSIONS: Goldenhar syndrome is a congenital condition, predominantly presenting as sporadic cases. Its diagnosis and management typically necessitate the involvement of multiple disciplines, including otolaryngology and craniofacial surgery. The syndrome encompasses a variety of craniofacial features, which can facilitate early diagnosis and guide subsequent therapeutic interventions.

Local injection of abaloparatide promotes mandibular condyle lengthening in adolescent rats via enhancing chondrogenesis and ossification.

BACKGROUND: Mandibular condylar hypoplasia negatively affects patient's facial appearance and dentofacial function. OBJECTIVE: To investigate the effect of local injection of the drug abaloparatide (ABL), an analogue of parathyroid hormone related protein (PTHrP), on promoting lengthening of the mandibular condyle. METHODS: Thirty adolescent male Sprague-Dawley rats were randomly divided into two groups, which received the injection of ABL or normal saline (the control) every 3 days in the temporomandibular joint (TMJ) cavity. Cone-beam computed tomography and immunohistochemistry assays were performed at 2, 4 and 6 weeks since the injection. Mandibular condylar chondrocytes (MCC) and pre-osteoblasts were treated with ABL or PBS, followed by the CCK-8 detection, IC50, real-time PCR assay, Western Blot and immunofluorescence staining. RESULTS: In vivo, compared with the control, the ABL group significantly increased the mandibular condylar process length (by 1.34 ± 0.59 mm at 6 weeks), the thickness of the cartilage layer, and enhanced the matrix synthesis. The ABL group had significant up-regulation of SOX 9, COL II, PTHrP and PTH1R, down-regulation of COL X in the cartilage, up-regulation of RUNX 2, and unchanged osteoclastogenesis in the subchondral bone. In vitro, the intra-TMJ injection of ABL promoted the MCC proliferation, with up-regulated expression of chondrogenic genes, and enhanced osteogenic differentiation of the pre-osteoblasts. CONCLUSIONS: Intra-TMJ injection of abaloparatide promotes mandibular condyle lengthening in the adolescent rats via enhancing chondrogenesis in the mandibular condylar cartilage and ossification in the subchondral bone.

Congenital Mandibular Hypoplasia: Patient-Specific Total Joint Replacement as a Line Extension in the Treatment of Complex Craniofacial Anomalies.

Introduction: Congenital mandibular hypoplasia (CMH) remains challenging because of the underlying combined hard and soft tissue deficiency. Treatment options include craniofacial distraction, orthognathic surgery, and autologous grafts, although the latter produces inadequate results after distraction and autologous grafting. Unsatisfactory long-term stability may cause relapse, necessitating reoperation. Material and Methods: We investigated the feasibility of using alloplastic total joint replacement (TJR) in growing and young adult CMH patients. The primary outcome was long-term reconstruction stability, without implant failure. Secondary outcomes were TMJ function and pain, and jaw movements achieved during surgery. Results: Three patients (age: 9-22 years) were treated by the same surgeon at one institution during 2018-2021. Anamnesis and clinical parameters were obtained from patient records. Preoperative 3D-scans were superimposed with postoperative 3D-scans and preoperative plans, including TJR-implant STL files, to measure jaw movement. All patients underwent prior reconstructive surgery. Mandibular movement of 16.4-20.1 mm in the sagittal direction was achieved. Post-TJR follow-up ranged from 24 to 42 months. No long-term complications occurred. At the latest follow-up, the maximal interincisal opening was between 21 and 40 mm, and all implants were functioning, without failure. Conclusion: In selected CMH cases, alloplastic TJR can deliver satisfactory medium-term results with predictable and stable outcomes, even in growing patients.

Early Outcomes and Risk Factors in Orthognathic Surgery for Mandibular and Maxillary Hypo- and Hyperplasia: A 13-Year Analysis of a Multi-Institutional Database.

BACKGROUND: Orthognathic surgery (OS) is a frequently performed procedure for the correction of dentofacial deformities and malocclusion. Research on OS is mostly limited to single-surgeon experience or single-institutional reports. We, therefore, retrospectively analyzed a multi-institutional database to investigate outcomes of OS and identify risk factors for peri- and postoperative complications. METHODS: We reviewed the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) database (2008-2020) to identify patients who underwent OS for mandibular and maxillary hypo- and hyperplasia. The postoperative outcomes of interest included 30-day surgical and medical complications, reoperation, readmission, and mortality. We also evaluated risk factors for complications. RESULTS: The study population included 674 patients, 48% of whom underwent single jaw surgery, 40% double jaw surgery, and 5.5% triple jaw surgery. The average age was 29 ± 11 years, with an equal gender distribution (females: n = 336; 50%, males: n = 338; 50%). Adverse events were relatively rare, with a total of 29 (4.3%) complications reported. The most common surgical complication was superficial incisional infection (n = 14; 2.1%). While the multivariable analysis revealed isolated single lower jaw surgery (p = 0.03) to be independently associated with surgical complication occurrence, it also identified an association between the outpatient setting and the frequency of surgical complications (p = 0.03) and readmissions (p = 0.02). In addition, Asian ethnicity was identified as a risk factor for bleeding (p = 0.003) and readmission (p = 0.0009). CONCLUSION: Based on the information recorded by the ACS-NSQIP database, our analysis underscored the positive (short-term) safety profile of OS. We found OS of the mandible to be associated with higher complication rates. The calculated risk role of OS in the outpatient setting warrants further investigation. A significant correlation between Asian OS patients and postoperative adverse events was found. Implementation of these novel risk factors into the surgical workflow may help facial surgeons refine their patient selection and improve patient outcomes. Future studies are needed to investigate the causal relationships of the observed statistical correlations.

Orthodontic-surgical treatment of an Angle Class II malocclusion patient with mandibular hypoplasia and missing maxillary first molars: A case report.

BACKGROUND: Adult patients presenting with Angle Class II division 1 malocclusions that have a strong skeletal etiology can be challenging for clinicians, particularly if accompanied by retrognathia of the mandible and a dolichofacial growth pattern. CASE SUMMARY: In this case report, we describe the successful orthodontic and surgical management of a 20-year-old woman with an Angle Class II malocclusion with a severe anteroposterior skeletal discrepancy characterized by mandibular deficiency. She had incompetent lips, dental and skeletal Class II malocclusion, high mandibular plane angle, mild mandibular crowding, and two missing maxillary first molars. The treatment plan comprised: (1) Extraction of two mandibular second premolars to decompensate and retract mandibular incisors; (2) pre-surgical alignment, leveling, and space closure of the teeth in both arches, and protraction of the second maxillary molars to close the maxillary space; (3) surgical treatment including a LeFort I osteotomy for maxillary retraction and rotation, a bilateral sagittal split osteotomy for mandibular advancement and rotation, and a genioplasty for correctting the skeletal deformities; and (4) post-surgical correction of the malocclusion. CONCLUSION: The patient's facial esthetics was significantly improved and a desirable occlusion was achieved after 16 mo treatment. Follow-up records after 2 years showed stable esthetics and function.

A Possible Incomplete Form of Treacher Collins Syndrome: A Case Report.

Treacher Collins syndrome (TCS) is a rare genetic disorder that affects craniofacial development due to malformation of the first and second branchial arches. The TCOF1 gene is mainly responsible for this condition. Here, we present a case of a 13-year-old adolescent girl with complaints of maligned teeth with conductive deafness. On clinical examination, she had retrognathia, a broad nose, maligned teeth, a high arch palate, and midfacial hypoplasia. On the basis of the clinical findings, a diagnosis of a mild-variant TCS was made as eyes were not involved and supportive treatment was given to the patient. The symptoms of the disease have a varying range of severity. Early diagnosis and supportive treatments, which include multidisciplinary treatment involving pediatrics, otolaryngologists, audiologists, orthodontists, and psychologists, are very important for the management of such cases.

A likely pathogenic POLD1 variant associated with mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome in a Chinese patient.

BACKGROUND: Mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome (MDPL; OMIM# 615381) is a rare autosomal dominant disorder, with only a few reported cases worldwide. Herein, we describe the clinical features and underlying molecular etiology of MDPL syndrome in an 8-year-old Chinese patient. METHODS: We performed otological, endocrine, ultrasound, and radiological examinations, as well as genetic testing. Additionally, the literature concerning MDPL was reviewed to do a retrospective analysis of the pathogenesis, genotype-phenotype correlation, and clinical management. RESULTS: The proband was diagnosed with MDPL, presenting with mandibular hypoplasia, a characteristic facial appearance, lipodystrophy, and sensorineural hearing loss (SNHL). Whole-exome sequencing and bioinformatics analysis revealed a de novo missense variant in the POLD1 gene, NM_002691.4:c.3185A>G (NP_002682.2:p.(Gln1062Arg)). The retrospective analysis showed wide variation in the MDPL phenotype, but the most frequent features included mandibular hypoplasia, characteristic facial appearance, lipodystrophy, and SNHL. CONCLUSIONS: This study supplements the mutational spectrum of POLD1. The genetic analysis contributes to the diagnosis of syndromic deafness, and it has a vital role in clinical management and future genetic consultation.

Mandibular stability and condylar changes following orthognathic surgery in mandibular hypoplasia patients associated with preoperative condylar resorption.

OBJECTIVES: To evaluate postoperative mandibular stability and condylar changes in patients with mandibular hypoplasia and preoperative condylar resorption (CR) undergoing orthognathic surgery. MATERIALS AND METHODS: Fifty-four patients were included in this retrospective study. Computed tomography (CT) scans were acquired preoperatively (T0), 2-7 days immediate postoperatively (T1), and at least 1 year postoperatively (T2). Three-dimensional (3D) cephalometric analysis and measurements of condylar angle, volume, and position (joint spaces) were performed. A 2-mm mandibular relapse was deemed clinically acceptable. We also analyzed the correlations between relapse and postoperative CR and susceptible factors using a multivariate logistic regression model. RESULTS: The results showed one year after the surgery, the average mandibular relapse was 1.0 mm (p < 0.05), and the average reduction of condylar volume was 152.4 mm3 (12.7%). Condyle-fossa relationships were improved immediately after the surgery, with a tendency of returning to their original state in the follow-up (p < 0.05). Anteroposterior advancement at point B (B-CP advancement) at T1 and superior joint space (SJS) at T0 were significantly correlated with mandibular relapse, and postoperative CR was mainly associated with vertical increasement at point B (B-AP increasement) at T1. The optimal cut-off values were as follows: 1.6 mm for SJS, 4.2 mm for B-CP advancement, and 1.8 mm for B-AP increasement. Concomitant advancement Genioplasty showed no significant correlation with relapse and postoperative CR. CONCLUSIONS: While patients with mandibular hypoplasia and preoperative CR were vulnerable to further condylar resorption after mandibular advancement, the treatment outcomes were generally clinically acceptable. Postoperative relapse was associated with a larger than 4.2 mm of mandibular advancement measured at B-CP and a larger than 1.6 mm of superior joint space measured at SJS, and postoperative CR was associated with a larger than 1.8 mm of mandibular vertical increasement measured at B-AP. CLINICAL RELEVANCE: The findings of this study suggested that the mandibular advancement might be limited to 5 mm for patients with preoperative CR. A concomitant advancement genioplasty might also be considered to achieve a better facial profile in these patients.

Orthopaedic Aspects of SAMS Syndrome.

The combination of short stature, auditory canal atresia, mandibular hypoplasia, and skeletal abnormalities (SAMS, OMIM: 602471) has been reported as an ultra-rare, autosomal-recessive developmental disorder with unique skeletal anomalies. To the present date, only four affected individuals have been reported. There are several striking orthopaedic diagnoses within the SAMS syndrome. In particular, the scapulohumoral synostosis and the bilateral congenital ventral dislocation of the hips. The purpose of this report is to underline the importance of recognizing pathognomic features of SAMS syndrome. Whenever a bilateral congenital ventral dislocation of the hips and/or a scapulohumoral synostosis is found or clinically suspected, SAMS syndrome should be considered as the primary diagnosis until proven otherwise.

Osteogenesis Modulation: Induction of Mandibular Bone Growth in Adults by Electrical Field for Aesthetic Purposes.

BACKGROUND: A new technique in plastic surgery termed Osteogenesis Modulation is described. This technique uses a surgically implanted, battery-operated medical device to deliver customized electrical pulses to produce mandibular bone growth. This device was designed to be a temporary, nonpermanent implant. The purpose of this study was to review both the safety and efficacy of Osteogenesis Modulation. METHODS: This study comprises two phases. Phase I involved experimental technology development and animal experiments. Phase II included technology development for clinical use and a clinical trial. In Phase II, four patients with a diagnosis of mandibular hypoplasia and microgenia underwent surgical implantation of the novel medical device over the chin bone. Once a satisfactory change of contour of mandibular bone was achieved, the devices were removed. In all patients, the devices were left in place for 12 months, then surgically removed under local anesthesia. Preoperative and long-term postoperative cephalometric controls were done. RESULTS: In all patients, symmetrical mandibular bone growth was observed with good-to-excellent aesthetic results. The overall follow-up period was 39 months. Cephalometric controls taken 3 to 6 months after the device removal showed an average increase in mandible length of 5.26mm (range, 2.83-7.60mm) CONCLUSIONS: Preliminary clinical results suggest that Osteogenesis Modulation is a safe, minimally invasive, and effective alternative treatment for the correction of mandibular hypoplasia in selected cases. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Whole-Exome Sequencing Reveals Rare Germline Mutations in Patients With Hemifacial Microsomia.

Hemifacial microsomia (HFM) is a rare congenital disease characterized by a spectrum of craniomaxillofacial malformations, including unilateral hypoplasia of the mandible and surrounding structures. Genetic predisposition for HFM is evident but the causative genes have not been fully understood. Thus, in the present study, we used whole-exome sequencing to screen 52 patients with HFM for rare germline mutations. We revealed 3,341 rare germline mutations in this patient cohort, including those in 13 genes previously shown to be associated with HFM. Among these HFM-related genes, NID2 was most frequently mutated (in 3/52 patients). PED4DIP, which has not been previously associated with HFM, exhibited rare variants most frequently (in 7/52 patients). Pathway enrichment analysis of genes that were mutated in >2 patients predicted the "laminin interactions" pathway to be most significantly disrupted, predominantly by mutations in ITGB4, NID2, or LAMA5. In summary, this study is the first to identify rare germline mutations in HFM. The likely disruptions in the signaling pathways due to the mutations reported here may be considered potential causes of HFM.

Accuracy of virtually planned mandibular distraction in a pediatric case series.

The aim of this study was to describe the utility of 3D technology in mandibular distraction (MD) for patients with mandibular hypoplasia (MH), using 3D-printed cutting guides (CGs), and to assess the differences between virtual surgical planning (VSP) and the final result. A descriptive retrospective study of five patients diagnosed with MH, who required unilateral or bilateral MD, was carried out between January 2018 and January 2020. All patients underwent preoperative craniofacial CT scan and a 3D VSP was executed. MD was performed with the help of the 3D-printed CG. Before removal of the distractor, another CT scan was performed to compare the actual final result with the VSP. A mean difference of <4° was found for the osteotomy direction, < 7° for distractor position, and <2 mm for posterior screw placement. VSP and 3D-printed CGs have revolutionized surgical planning, facilitating surgical treatment and improving the final result. In our sample, the variations in osteotomy line, distractor position, and posterior screw placement have been minor, making the outcome more predictable.

Infant mandibular distraction in absence of ascending ramus: case series.

BACKGROUND: Severe microretrognathia with the absence of ascending mandibular ramus is a challenging deformity and treatment must aim to avoid tracheostomy or remove it as soon as possible. Although it is not often reported, mandibular distraction osteogenesis represents a valid treatment option in infants affected by hypoplastic mandible Pruzansky-Kaban type IIb and III. CASE PRESENTATION: The authors describe 3 cases of infants affected by severe respiratory insufficiency due to congenital mandibular hypoplasia, with follow up ranging from 4 to 8 years. Clinical and technical considerations on treatment choices and outcomes are discussed starting from review of the literature and direct clinical experience. CONCLUSION: Early mandibular distraction, specifically bidirectional distraction, is an effective and repeatable technique that leads to mandible lengthening with counterclockwise rotation, pogonion projection increase, anteropositioning of the tongue base, and expansion of oropharyngeal volume with positive effect on the respiratory problems of the infant. Even in Treacher Collins patients, known to have a low decannulation rate, all of these elements are essential for effective speech and swallowing therapy and for a subsequent attempt of decannulation.

Idiopathic Mandibular Condyle Resorption.

Idiopathic mandibular condylar resorption is a rare condition in which the mandibular condyle of the temporomandibular joint (TMJ) becomes resorbed and thus reduces in size and volume. This leads to TMJ dysfunction that commonly requires surgical correction; however, more conservative interventions can also be utilized. We present a case of idiopathic mandibular condyle resorption in a 17-year-old female presenting with TMJ pain and clicking with mastication. A definitive diagnosis of this condition ultimately requires imaging studies, a reliable option being magnetic resonance imaging (MRI), which will reveal erosion of the mandibular condylar process (often bilaterally) with diminished mass and volume leading to the known sequelae of symptoms.

A Novel Syndrome With Short Stature, Mandibular Hypoplasia, and Osteoporosis May Be Associated With a PRRT3 Variant.

CONTEXT: Despite considerable progress in elucidating the molecular basis of various progeroid syndromes, some rare patients remain unexplained. OBJECTIVE: To elucidate molecular genetic basis of a novel autosomal recessive progeroid syndrome. PARTICIPANTS: A 24-year-old male and his 18-year-old sister with short stature, mandibular hypoplasia, pointed nose, shrill voice, severe osteoporosis, and short eyebrows and their unaffected siblings and parents belonging to a consanguineous Arab family. RESULTS: Using exome and Sanger sequencing, we report a novel homozygous p.Glu394Lys disease-causing variant in proline-rich transmembrane protein 3 (PRRT3). PRRT3 belongs to the family of proline-rich proteins containing several repeats of a short proline-rich sequence, but its function remains to be determined. Preliminary observations showing colocalization of Prrt3 and synaptophysin support its role in vesicle exocytosis. Consistent with the highest messenger ribonucleic acid expression of PRRT3 in the pituitary, both the patients had mild growth hormone deficiency but had near normal reproductive development. CONCLUSIONS: We conclude that the homozygous p.Glu394Lys variant in PRRT3 may be associated with a novel autosomal recessive, progeroid syndrome with short stature, mandibular hypoplasia, osteoporosis, short eyebrows, and mild growth hormone (GH) deficiency. Our findings extend the spectrum of progeroid syndromes and elucidate important functions of PRRT3 in human biology, including secretion of GH from the pituitary.

Effects of nutrition and hormones on functional appliance treatment outcome in patients with skeletal Class II malocclusion.

BACKGROUND: Functional appliances has been used for treatment of skeletal Class II malocclusion for a long time; however, the real skeletal effects, mandibular growth particularly, remain insufficient. Several auxiliary approaches have been attempted with the hope of enhancing treatment effects. In this review, we summarize and discuss the use of additional nutrition and hormones to assist the functional appliance treatment on patients with skeletal Class II malocclusion. METHODS: Relevant articles were identified by electronic research in MEDLINE Ovid using keywords such as "nutrition," "hormone," "functional appliance," "orthodontics," "maxillofacial development," and "maxillofacial abnormalities." References of related articles were assessed for relevant studies to identify additional published references. RESULTS: The literature search yielded 239 studies. According to the current literature, use of additional nutrition and hormones, including growth hormones, sex hormones, insulin, and insulin-like growth factor I, seem to improve the effects of functional appliance treatment on patients with skeletal Class II malocclusion. CONCLUSIONS: The current evidence indicates that additional nutrition or hormones might improve the treatment effects on mandibular hypoplasia compared with the functional appliance alone, which is a promising approach and calls for further studies.

Efficacy and Versatility of Intraoral Monoplanar Distractors in the Correction of Extreme Mandibular Deformities and Severe Facial Asymmetries - A Retrospective Study.

INTRODUCTION: Distraction osteogenesis, has emerged as a revolutionary concept and an effective means to treat extensive craniomaxillofacial defects and malformations. In cases of mandibular distraction for patients with extreme acquired mandibular deformities, there is so far, no recorded literature on precise quantification and scientific estimation of the percentage of the efficacy of intraoral monoplanar distractors, for an objective evaluation of their effectiveness. The objective was to study the efficacy of intraoral mandibular monoplanar distractors in the correction of severe acquired mandibular deformities and gross facial asymmetries. To objectively evaluate their effectiveness by calculating the percentage of distraction achieved as against that aimed for, and to evaluate early and late complications encountered with their use. MATERIALS AND METHODS: Five patients in the age group of 10-22 years, with extreme mandibular deformity and gross facial asymmetry secondary to longstanding temporomandibular joint (TMJ) ankylosis, were treated. Four patients had unilateral and one patient had bilateral TMJ ankylosis, with varying degrees of acquired mandibular hypoplasia, retrogenia, retrognathia, and asymmetry. They were managed by unilateral (3 patients) and bilateral (2 patients) mandibular distraction. RESULTS: Maximum horizontal corpus and vertical Ramal distraction achieved was 19 mm and 17.6 mm, respectively. The percentage of the efficacy of the intraoral monoplanar distractors used in this study ranged from 65.38% to 109.09%. Severe mandibular deformities and facial asymmetries were successfully corrected in all five patients, with no major early or late complications encountered in any of them. Results achieved were stable with nil incidence of relapse. DISCUSSION: Estimation of percentage of the efficacy of distraction achieved, helped in objectively evaluating the effectiveness of the intraoral mandibular distractors. A low complication rate and good esthetic and functional outcomes achieved in all the patients demonstrated the reliability of this treatment modality in the management of extreme mandibular deformities and severe facial asymmetries.