ABSTRACT Background Cleft lip and palate (CLP) is the most prevalent craniofacial malformation, frequently associated with maxillary hypoplasia, velopharyngeal dysfunction and aesthetic alterations that compromise quality of life. Among surgical strategies, orthognathic surgery (OS) and distraction osteogenesis (DO) remain the most widely applied techniques for correction of maxillary deficiencies in CLP patients. Objective This literature review focuses on analysing and synthesising the current evidence on the most commonly used OS and DO techniques in patients with CLP, highlighting their indications, results and limitations. Methods A systematic search was conducted in PubMed, Scopus, Cochrane Library and Taylor and Francis, including clinical studies, systematic reviews and meta‐analyses published in the last 15 years. Results OS provides immediate correction of moderate maxillofacial deformities and improves occlusion and facial harmony, but carries a considerable risk of relapse and velopharyngeal dysfunction. In contrast, DO enables gradual skeletal advancement with superior long‐term stability and better soft tissue adaptation, though it requires prolonged treatment and tolerance to distraction devices. Recent evidence indicates that hybrid and segmental distraction techniques may further enhance stability and reduce complications. Conclusion The choice between OS and DO should be individualised according to the severity of maxillary hypoplasia, patient age, and functional‐aesthetic objectives. While OS remains effective for moderate corrections, DO demonstrates advantages in severe cases. Multicenter studies with standardised protocols are needed to strengthen long‐term evidence and optimise surgical decision‐making in CLP management.

IntroductionManagement of cleft maxillary hypoplasia (CMH) is complex and influenced by multiple factors. While orthognathic surgery (OGS) remains a standard corrective approach, large maxillary advancement often result in compromised stability. Alternative methods, such as anterior maxillary distraction (AMD) and Le Fort 1 distraction, offer gradual advancement but may not address CMH in all dimensions. This study assesses the efficacy of staging CMH treatment using both AMD and OGS.DesignRetrospective Observational Study.PatientsUnilateral cleft lip and palate patients with at least 1 year of follow-up post-CMH correction were included.InterventionPatients were grouped by maxillary arch perimeter (MAP) and intervention: Group A (<80 mm, AMD + OGS), Group B (>90 mm, OGS only), and Group C (<80 mm, OGS only). Maxillary arch perimeter changes, OGS movement, relapse, and postoperative orthodontic treatment duration were evaluated.ResultsFifteen patients (5 per group) were included. Group A showed increased MAP by 7.84 mm post-AMD, accommodating palatally placed teeth. Mean maxillary movement during OGS was comparable in Group A (7.4 mm) and Group B (7.5 mm) but greater in Group C (11.8 mm). Relapse was the least in Group B, followed by Group A and then C. Postoperative orthodontic duration varied: 4 to 6 months for Group A, 3 to 5 months for Group B, and 12 to 16 months for Group C.ConclusionAnterior maxillary distraction effectively addresses crowding, maintains Bolton's ratio, increases MAP, and reduces the quantum of movement during subsequent OGS. A preoperative MAP ≥ 85 mm allowed good interarch intercuspation improving stability during OGS and reduced postoperative orthodontic duration. Anterior maxillary distraction proves to be a valuable interim procedure, enhancing overall treatment outcomes in CMH correction.

ObjectiveTo compare pharyngeal airway changes after anterior maxillary distraction and conventional maxillary distraction in patients with unilateral cleft lip and palate (UCLP).DesignRetrospective study.SettingTertiary care medical center for cleft care.PatientsA total of 20 patients with UCLP and hypoplastic maxilla were selected.InterventionGroup 1 (n = 10): Anterior Maxillary Distraction, Group 2 (n = 10): Conventional maxillary distraction.Outcome MeasuresThe primary outcomes assessed on pre- and postsurgical cone beam computed tomography were changes in the nasopharyngeal, oropharyngeal, and total pharyngeal airway area and volume, as well as the minimal axial area. These 2 distraction techniques were compared for their impact on upper airway dimensions.ResultsIn Group 1, the nasopharyngeal area increased significantly by 43.60 mm2, and the oropharyngeal area decreased by 40.80 mm2. There were no significant changes in the volume and area of all the other pharyngeal parameters. In Group 2, the nasopharyngeal area and volume increased by 202.70 mm2 and 5.0 cm3, respectively, the oropharyngeal area and volume increased by 176.20 mm2 and 7.9 cm3, respectively, the total pharyngeal area and volume increased by 380.50 mm2 and 14.0 cm3, respectively, and the minimal axial area increased by 133.50 mm2.ConclusionAnterior maxillary distraction had a negligible impact on the pharyngeal airway compared to conventional distraction. The choice between these 2 interventions should also take into consideration associated comorbidities related to pharyngeal airway and speech.

Introduction: Patients with cleft lip and palate often present maxillary hypoplasia owing to early surgical scarring, necessitating surgical advancement to restore facial balance and functional occlusion. Maxillary distraction osteogenesis, particularly with internal devices, is an effective technique for advancing the maxilla and surrounding soft tissues in patients with cleft lip and palate. These patients often present with complex jaw deformities requiring a multidisciplinary approach. Case Report: A 43-year-old male with unilateral cleft lip and palate presented with facial asymmetry and malocclusion. A multidisciplinary team performed maxillary distraction osteogenesis using an internal device, sagittal split ramus osteotomy, and plastic surgery. Maxillary distraction osteogenesis achieved maxillary advancement, clockwise rotation, and rolling. Subsequently, genioplasty and structural rhinoplasty were performed. The patient was satisfied with skeletal improvements and stable occlusion. Conclusion: A middle-aged patient with unilateral cleft lip and palate, who required maxillary distraction osteogenesis was successfully treated through close collaboration among multiple professionals. Maxillary distraction osteogenesis effectively corrects maxillary malposition by enabling three-dimensional movement of the maxilla. A multidisciplinary team approach is crucial for addressing jaw deformity.

Treatment Of Severe Maxillary Hypoplasia In Deciduous Dentition

Discussion: Association of Sphincter Pharyngoplasty and Long-Term Maxillary Hypoplasia in Patients with Cleft Palate.

Cleft maxillary hypoplasia (CMH) is a common sequela in patients with cleft lip and palate (CLP) who have undergone surgical and orthodontic interventions. Le Fort I maxillary distraction osteogenesis (DO) using a halo-type external distraction device is a well-established technique for maxillary advancement. However, simple anterior advancement may not yield satisfactory outcomes in cases involving severe segmental deviations. The authors reported our success in utilizing a combination of various maxillary DO and bone transport techniques to correct severe, complicated CMH. The patient was a 13-year-old girl with Angle Class III malocclusion and a history of congenital left CLP. She had undergone prior corrective surgeries, including cheiloplasty at 3 months of age, palatoplasty at 14 months, and rhinoplasty at 5 years. Our approach achieved favorable functional and aesthetic outcomes by addressing the complex deformities between maxillary segments. This case highlights the effectiveness of various DO strategies in managing severe, complicated CMH.

Patients with severe maxillary deficiency present a significant challenge for maxillofacial surgeons. These individuals often exhibit hypoplastic maxillae in all 3 spatial planes, frequently associated with dentofacial deformities such as cleft lip and palate. Midface hypoplasia has traditionally been treated with orthognathic surgery. However, this procedure has limitations, including the potential for relapse. To overcome these limitations, distraction osteogenesis with histiogenesis has emerged as a viable alternative for midfacial advancement. Consequently, distraction osteogenesis has become the surgical method of choice for patients with severe midface retrusion and maxillary-mandibular discrepancies >20mm. This paper aims to present the authors' clinical experience using this surgical approach.

Comparison of treatment effects with or without miniscrews for maxillary protraction in growing patients with Class III malocclusion.

Anatomical insights into midface retrusion and maxillary hypoplasia in adult cleft lip and palate patients post-skeletal maturity.

Apert syndrome is a rare congenital disorder first noted by Wheaton in 1894 and more precisely described by Eugene Apert in 1906. It is primarily characterized by craniosynostosis, midfacial hypoplasia, and complex syndactyly of the hands and feet, along with various systemic and oral abnormalities. The syndrome is estimated to occur in approximately 1 in 1,60,000 live births and typically follows an autosomal dominant inheritance pattern. Most cases are associated with spontaneous mutations in the fibroblast growth factor receptor 2 (FGFR2) gene, which plays a crucial role in bone development and tissue formation. This case report details the clinical and dental management of a 4-year-old female who was diagnosed with Apert syndrome at birth. The patient presented with hallmark craniofacial features such as a high, prominent forehead, flat midface, syndactyly affecting both hands and feet, and notably delayed developmental milestones. She had a history of undergoing multiple surgical interventions, including cranial vault reconstruction to alleviate intracranial pressure and syndactyly release procedures to improve hand functionality. Intraorally, the patient showed maxillary hypoplasia, a high-arched palate, delayed eruption of primary teeth, dental crowding, and multiple carious lesions. Dental care was tailored to her special healthcare needs, utilizing the silver-modified atraumatic restorative technique (SMART), which involves the use of silver diamine fluoride and glass ionomer cement to arrest caries and restore teeth in a minimally invasive manner. A preventive oral health plan, including dietary counselling, fluoride varnish application, and regularly scheduled recall visits, was instituted to maintain long-term dental and overall well-being.

Is Palatal Transmucosal Osteotomy With Piezoelectric Device Associated With Favorable Pterygomaxillary Junction Separation in Le Fort I Osteotomy?

Distraction Osteogenesis for Cleft Maxillary Hypoplasia: A Comparative Case Series of Four Surgical Planning Approaches

Accuracy of Virtual Surgical Planning in Managing Maxillary Hypoplasia in Adults with Cleft Lip &amp; Palate

Treatment of patients with maxillary hypoplasia caused by congenital cleft lip and palate (CLP) is still urgent up to date. Maxillary advancement using the distraction method is generally accepted, but the stability of this procedure remains controversial. Objective. To analyze and summarize the published materials on the assessment of stability of maxillary hypoplasia treatment using the distraction method in patients with CLP. Material and methods. A search for publications on the selected study topic in the MEDLINE and PubMed electronic databases was performed. The search was done by the following keywords: «cleft lip and palate», «maxillary hypoplasia», «distraction osteogenesis», «stability», «relapse», «RED», «intraoral distraction device» in Russian and English. Results. The study included 13 publications, and most of them showed stable results of treatment using the distraction method. The relapse rate was lower in the distraction osteogenesis group than in the traditional orthognathic surgery group after 5-year follow-up. Long-term stability of maxillary advancement after orthognathic surgery was higher in studies on the application of internal distractors than in the use of RED device. Conclusion. Maxillary advancement in patients with CLP using distraction method is a stable treatment method.

INTRODUCTION/BACKGROUNDPycnodysostosis is a rare skeletal dysplasia and a subtype of osteopetrosis caused by deficient activity of the lysosomal protease cathepsin K (CTSK) gene. We present a case of pycnodysostosis exhibiting characteristic clinical features alongside a fragility fracture—an association that has been rarely reported. CASEA 33-year-old Malay female, born of a consanguineous marriage, presented with a subtrochanteric fracture of the proximal left femur following a trivial fall and underwent open reduction with locking compression plate fixation. She was noted to have a short stature (height: 145 cm). Further examination revealed frontal bossing, maxillary hypoplasia, and brachydactyly of both hands and feet. Other systemic examinations including neurological assessment were unremarkable. A family history of similar physical traits was noted in her late paternal grandmother, though medical records were unavailable. Her elder sister exhibited similar abnormalities. Laboratory investigations showed a low insulin-like growth factor 1 (IGF-1) level, but her insulin tolerance test was normal, excluding growth hormone deficiency. Other blood parameters, including complete blood count, serum calcium, phosphate, 25-hydroxy vitamin D, alkaline phosphatase, thyroid function tests, and cortisol were within normal limits. Bone mineral density (BMD) analysis revealed an elevated Z-score of +5.5 at the total hip and +0.9 at L1-L4. Genetic analysis identified a homozygous variant of uncertain significance in the CTSK gene, which is associated with autosomal recessive pycnodysostosis. Given the lack of a specific treatment, symptomatic management was initiated, focusing on fracture prevention, oral hygiene, and psychological support. CONCLUSIONThis case underscores the need to consider rare genetic skeletal dysplasias like pycnodysostosis in individuals presenting with fragility fractures, distinct craniofacial and skeletal features, and unusually high bone mineral density. Prompt recognition, aided by genetic testing, is crucial for guiding patient counseling, monitoring, and coordinated multidisciplinary care. Although no curative treatment exists, early diagnosis can help reduce complications and support a better quality of life.

BackgroundSegmental Le Fort I osteotomy combined with anterior maxillary clockwise rotation has been proposed as an effective treatment for maxillary hypoplasia. However, the stability of maxilla after the operation remains unknown.MethodsA total of 30 patients undergoing segmental Le Fort I osteotomy were retrospectively included. The follow-up period was more than one year. The stability of anterior maxilla after clockwise rotation was evaluated by cone beam computed tomography (CBCT) performed before surgery (T0), three days after surgery (T1), and at least one year after surgery (T2), respectively. The key parameters were the postoperative relapse of the anterior maxillary clockwise rotation angle (CRA) and paranasal advancement.ResultsFollowing segmental Le Fort I osteotomy, the average CRA of the anterior maxilla was 10.02° ± 3.86°, while the mean paranasal advancement was 6.22 ± 1.40 mm. At the one-year follow-up, the relapse of CRA and paranasal advancement were -0.42° ± 2.51° (p = 0.951) and -0.28 ± 0.83 mm (p = 0.08), respectively, suggesting good postoperative stability. Additionally, no significant correlation was found between the intraoperative CRA and its relapse over time.ConclusionSegmental Le Fort I osteotomy combined with anterior maxillary clockwise rotation demonstrates favorable stability up to one year postoperatively, making it a reliable approach for the treatment of maxillary hypoplasia.

Abstract Introduction Crouzon’s syndrome is caused by fibroblast growth factor receptor 2 (FGFR2) gene mutation and is seen in 16 out of 1 million live births. It is characterized by the premature closing of calvarial and cranial base sutures as well as the orbit and maxillary complex resulting in exophthalmos, flattened forehead, midface hypoplasia, and hypoplastic maxilla. This deformity, this leads to a high prevalence of obstructive sleep apnea. Here we present a case of the progression of OSA in a patient with Crouzon’s with subsequent medical and surgical interventions. Report of case At age 15 months, patient was evaluated by Pediatrician for loud snoring and witnessed apneas. Her I’M SLEEPY questionnaire was positive with score of 4 and physical exam was pertinent for +3 tonsillar hypertrophy. She was evaluated by ENT where evaluation of pathognomonic facies was initiated and found positive for Crouzon’s due to FGFR2 gene variant - c.1018T&amp;gt;C, p.Tyr340His. At 2 years old, initial PSG showed AHI: 24.3, RDI: 24.3, Oxygen Nadir 93%. Underwent T&amp;A with ENT and established care with Sleep Medicine for management. Found to have continued symptoms of loud snoring, apneas, unrefreshing sleep. Second PSG at 4 years old, showed AHI 8.8, RDI: 9.7, Oxygen Nadir 93%. Attempted usage of AutoPAP, unable to tolerate due mask intolerance and chronic rhinitis. Underwent anterior cranial vault reconstruction with midface advancement with OMFS and Neurosurgery. Third PSG at 6 years old, AHI 3.6. Compliance was difficult to implement due to mask intolerance. At 8 years old, underwent LeFort III surgery and corrective orbital surgery. Fourth PSG at 9 years old, AHI 4.1, RDI 16.5, Oxygen Nadir 97%. Recommendations to attempt AutoBiPAP and continued follow up with OMFS for surgical versus orthodontic interventions. Conclusion Crouzon’s syndrome is characterized with anatomical deformities which leads to high prevalence of OSA of 68% but data is limited given the overall epidemiology of this syndrome. The predominant contributing factor for OSA is midface hypoplasia. Treatment options include T&amp;A followed by PAP using nasal interface and definitive treatment is surgical midface advance like Le Fort III with distraction osteogenesis, monobloc advancement and facial bipartition. Support (if any)

Abstract Introduction Pediatric OSA has been linked with cognitive impairments, behavioral effects leading to academic underperformance and significant cardiovascular and metabolic consequences. Craniofacial abnormalities are one of the contributing factors for an increased risk of OSA and a cause of relapse or incomplete resolution of OSA. Orthodontic treatments have emerged as one of the treatment options for patients with craniofacial abnormalities. Report of case A 17-year-old patient with a history of mild obstructive sleep apnea (OSA) diagnosed at age 10 presented with persistent sleep-disordered breathing (SDB). Symptoms began at age 8, including snoring and loud breathing. The patient underwent adenoidectomy at 6 years of age and tonsillectomy at 10 years of age, with persistent symptoms leading to referral to Sleep Medicine and ENT. Physical examination findings included Class II malocclusion, left posterior crossbite, high-arched palate, macroglossia, maxillary and mandibular hypoplasia with lip pursing, a right-sided deviated nasal septum with internal nasal valve collapse, and bilateral inferior turbinate hypertrophy. A CT sinus study revealed bilateral concha and a septal spur on the right. Despite offering surgical intervention with septorhinoplasty and turbinate reduction, the patient and mother opted against proceeding with surgery. A repeat split-night polysomnography study demonstrated progression to severe OSA, with an apnea-hypopnea index (AHI) of 10.7 and an oxygen nadir of 82%. CPAP titration identified an optimal pressure of 7 cm H2O, resulting in a residual AHI of 0. To address structural issues, the patient established care with an orthodontist and an oral maxillofacial surgeon. Orthodontic treatment with braces and RME was initiated, with an expected duration of approximately two years to optimize the craniofacial structure and improve airway patency. Meanwhile, the patient was bridged with auto CPAP (settings: 5-15 cm H2O), which was well-tolerated and demonstrated adequate compliance. Follow-up visits noted significant improvement in residual symptoms of SDB. Conclusion Rapid Maxillary Expansion (RME) can lead to optimal outcomes with early detection and timely intervention. RME increases maxillary width, influences the dimensions of nasal vault, and increases upper airway space, with improvements in PSG parameters observed after one year of treatment. Support (if any)

P54. Increasing Number of Cleft-related Surgeries Results in Worsened Maxillary Hypoplasia: A Cephalometric Study