Maxillary protraction in adult cleft lip and palate by a rigid external distraction device with dentoskeletal anchorage

Objective: The objective is to evaluate the effects of maxillary distraction osteogenesis (DO) in an adult patient with unilateral cleft lip and palate (UCLP) by using a rigid external distraction (RED) device with dentoskeletal anchorage. Method: 31-year-old male patient with UCLP with severe maxillary hypoplasia, dolichofacial growth pattern, negative overjet and 1.5 mm openbite. After pre-surgical orthodontic treatment, an intra-oral appliance was modified to prevent extrusion of the molars and clockwise rotation of the mandible. Stainless steel plates were soldered bilaterally to the intra oral appliance at the level of canines. During surgery, miniplates were inserted in the maxillary segment and fixed to the plates of the intra oral appliance with screws. Results: The mean distraction length was 12 mm immediately after DO. SNA increased from 73o to 82o after distraction. A significant advancement of the maxilla and correction of the sagittal Class III skeletal relationship was achieved. The vertical position of the mandible and the face was kept stable, and the soft tissue profile became more balanced. Conclusion: This intra oral appliance design achieved desired skeletal changes during maxillary protraction with RED device in dolichofacial CLP patient. Occlusion and facial profile changes was found to be stable in 1-year follow-up.PubMe

Treatment of A Skeletal Class III Patient with Maxillomandibular Advancement Surgery - Case Report

A 19-year-old female patient with skeletal Class III malocclusion, a great number of missing teeth, hyperactive mentalis muscle, and a severely concave facial profile presented for treatment. Treatment objectives were to align the arches, restore the gingival health in the mandibular anterior region, and achieve better facial esthetics. A lip release procedure was undertaken to lower the forces exerted by the mentalis muscle to the lower incisor teeth. Gingival grafts were placed before the surgery to restore and maintain the attached gingiva. Maxillomandibular advancement surgery as well as genioplasty to decrease the chin projection which would increase as a result of mandibular advancement was performed. Active treatment time was 33 months. Posttreatment evaluation revealed that most of the treatment objectives had been achieved. In this case report, the use of lip release and free gingival grafting procedures as an adjunct to orthognathic treatment is presented

Treatment of Cleft lip & Palate Related Maxillary Hypoplasia with RED System: Case Report

Clefts of the lip and palate present one of the most challenging congenital disorders to the orthodontists and craniofacial surgeons. A common finding is maxillary hypoplasia. Current case report presents the treatment of a 19 years old female unilateral cleft lip and palate patient characterized with maxillary constriction, retrusion and severe underjet. The case has been successfully treated with fixed appliance therapy and rigid external distraction and relapse at the end of treatment is minimal. However, longer follow-ups are required to assess the exact amount of stability

Reconstruction of mandibular defects using autografts combined with demineralized bone matrix and cancellous allograft

WOS: 000243853800018PubMed: 17251844Facial bone defects can be managed by a variety of means, such as inlay or onlay applied nonvascularized bone grafts, local pedicled/distal free osteocutaneous/osseous flaps or distraction osteogenesis. Although each method has its own advantages and drawbacks, the indications of using these methods may overlap in certain clinical situations. The use of nonvascularized bone grafts, conceptually and technically simple method, relies on creeping substitution of applied graft with regenerating new bone. However, using vascularized osteocutaneous free flaps provide not only plenty of bony tissue for reconstruction, but also soft tissue coverage over the reconstructed bony segment. Furthermore, bone healing in vascularized osteocutaneous free flaps resembles as the physiologic fracture healing. In selected cases with enough soft tissue coverage over the defective bony segment, on the other hand, using larger nonvascularized bone grafts with osteoinductive growth factors may decrease the risk of graft failure while avoiding the drawbacks of the free tissue transfer. Here we present our results of three cases with facial bone defects, to which nonvascularized iliac bone grafts and demineralized bone matrix with cancellous allograft (Orthoblast II (TM)) as a source of BMP were used together for mandibular reconstruction

Ortognatik cerrahi girişim geçirmiş hastaların iskelet sonuçlarının sefalometrik analizi

Ortognatik cerrahi girişim yapılmış 24 olgu, geç dönem iskelet sonuçlan, relaps ve sonucu olumsuz etkileyebilecek nedenler açısından değerlendirildi. İskelet sonuçlarının analizi, ameliyattan iki hafta öncesi alınmış, ameliyattan bir hafta sonra alınmış ve ameliyattan en az altı ay sonra alınmış yan sefalogramlarının karşılaştırmasına dayalı olarak yapıldı. Sefalometrik inceleme sonuçlarının istatistiksel analizi girişim şekline göre ayrılmış gruplar için ayrı olarak yapıldı. Mandibula geriletilen olgularda ortalama %14.2, mandibula ilerletilen olgularda ortalama %24 oranında iskelette relaps saptandı. 14 olguluk mandibula geriletilmesi olgu grubunda temporomandibular eklem sorunu, diş kapanış bozukluğu, girişim şekli, kullanılan tespit yönteminin relaps ile ilişkisi incelendi. Mandibula geriletilen olgularda yapılan tel ya da plakla tespitin relaps oranını azaltmakla beraber istatistiksel olarak relapsı etkilemediği sonucuna varıldı. Bimaksiller girişimlerin mandibula geriletilen olgularda relaps üzerinde fark oluşturmadığı görüldü. Böylece ortognatik cerrahi girişimlerde iskelette elde edilen sonucun değişebileceği ortaya konularak bu konuda etkili olabilecek girişim şekli, tespit yöntemi, diş kapanış bozukluğu ve eklem sorunu olması gibi faktörlerin relaps açısından önemi gözden geçirildi

Mutations In The Interleukin Receptor Il11Ra Cause Autosomal Recessive Crouzon-Like Craniosynostosis

We have characterized a novel autosomal recessive Crouzon-like craniosynostosis syndrome in a 12-affected member family from Antakya, Turkey, the presenting features of which include: multiple suture synostosis, midface hypoplasia, variable degree of exophthalmos, relative prognathism, a beaked nose, and conductive hearing loss. Homozygosity mapping followed by targeted next-generation sequencing identified a c.479+6T>G mutation in the interleukin 11 receptor alpha gene (IL11RA) on chromosome 9p21. This donor splice-site mutation leads to a high percentage of aberrant IL11RA mRNA transcripts in an affected individual and altered mRNA splicing determined by in vitro exon trapping. An extended IL11RA mutation screen was performed in a cohort of 79 patients with an initial clinical diagnosis of Crouzon syndrome, pansynostosis, or unclassified syndromic craniosynostosis. We identified mutations segregating with the disease in five families: a German patient of Turkish origin and a Turkish family with three affected sibs all of whom were homozygous for the previously identified IL11RA c.479+6T>G mutation; a family with pansynostosis with compound heterozygous missense mutations, p.Pro200Thr and p.Arg237Pro; and two further Turkish families with Crouzon-like syndrome carrying the homozygous nonsense mutations p.Tyr232* and p.Arg292*. Using transient coexpression in HEK293T and COS7 cells, we demonstrated dramatically reduced IL11-mediated STAT3 phosphorylation for all mutations. Immunofluorescence analysis of mouse Il11ra demonstrated specific protein expression in cranial mesenchyme which was localized around the coronal suture tips and in the lambdoidal suture. In situ hybridization analysis of adult zebrafish also detected zfil11ra expression in the coronal suture between the overlapping frontal and parietal plates. This study demonstrates that mutations in the IL11RA gene cause an autosomal recessive Crouzon-like craniosynostosis.PubMedScopu

Alx4 Dysfunction Disrupts Craniofacial and Epidermal Development

Genetic control of craniofacial morphogenesis requires a complex interaction of numerous genes encoding factors essential for patterning and differentiation. We present two Turkish families with a new autosomal recessive frontofacial dysostosis syndrome characterized by total alopecia, a large skull defect, coronal craniosynostosis, hypertelorism, severely depressed nasal bridge and ridge, bifid nasal tip, hypogonadism, callosal body agenesis and mental retardation. Using homozygosity mapping, we mapped the entity to chromosome 11p11.2-q12.3 and subsequently identified a homozygous c.793C -> T nonsense mutation in the human ortholog of the mouse aristaless-like homeobox 4 (ALX4) gene. This mutation is predicted to result in a premature stop codon (p.R265X) of ALX4 truncating 146 amino acids of the protein including a part of the highly conserved homeodomain and the C-terminal paired tail domain. Although the RNA is stable and not degraded by nonsense-mediated RNA decay, the mutant protein is likely to be non-functional. In a skin biopsy of an affected individual, we observed a hypomorphic interfollicular epidermis with reduced suprabasal layers associated with impaired interfollicular epidermal differentiation. Hair follicle-like structures were present but showed altered differentiation. Our data indicate that ALX4 plays a critical role both in craniofacial development as in skin and hair follicle development in human.WoSScopu