Maxillary Hyperplasia Research Articles

Does the Rigid External Distraction Device Alter Maxillary Pitch in Cleft Maxillary Distraction?

Statement of problemCorrection of cleft maxillary hypoplasia remains a controversial topic. Surgical correction can be accomplished through Le Fort I orthognathic correction, internal distraction osteogenesis, or external distraction osteogenesis.1 Proponents of rigid external distraction (RED) osteogenesis describe the ability of multi-vector directional distraction control as opposed to univector internal distraction. There are currently limited data on the ability of the RED system to alter the maxillary occlusal pitch.The purpose of this study is to address the following clinical question: “Among cleft patients presenting with severe maxillary hypoplasia and treated with rigid external distraction, does the rigid external distraction system result in alteration of the maxillary pitch?”The study hypothesis is: among cleft patients presenting with maxillary hypoplasia treated with RED, the investigators hypothesize that there is no change in maxillary pitch with distraction.The study's specific aims are to design a retrospective cohort study and enroll a sample composed of cleft patients with maxillary hypoplasia treated with RED: 1) identify a cohort of patients with cleft maxillary hypoplasia, 2) quantify the degree of pre-operative maxillary hyperplasia, 3) measure changes in maxillary position achieved through RED. Materials and methodsTo address the research objectives, the investigators designed a retrospective cohort study. The study sample was derived from the population of patients with cleft maxillary hypoplasia who underwent RED by the Department of Oral and Maxillofacial Surgery at the University of Alabama - Birmingham from June 2007 to August 2019. Subjects eligible for study inclusion had cleft maxillary hypoplasia and underwent RED. Subjects were excluded if there were incomplete data collection or their records were not available for review.Demographic and radiographic variables were recorded. Demographics included gender, age at date of surgery, and history of ULCP vs. BLCP. Radiographic variables included standard cephalometric data recorded prior to distraction (T1) and after completion of distraction (T2). Methods of data analysisDescriptive statistics were computed for all study variables. Due to the potential for both positive and negative occlusal pitch change, a paired 2-tailed T-test was used to compare T1 and T2. The level of statistical significance was set at P < .05. ResultsThe study sample was composed of 45 subjects with a mean age at surgery of 12.2+3.4 years. Twenty-two (49%) were BCLP and 27 (60.0%) were male. At T1, mean SNA was 76.35+/-6.4 degrees, and the maxillary occlusal plane to Frankfort horizontal plane was 8.18+/-6.9 degrees. The mean anterior-posterior distraction distance was 11.4+/-5.7 mm. At T2, the mean SNA was 86.0+/-7.5 degrees, and the occlusal plane to FH was 3.78+/-7.3 degrees. The average maxillary occlusal pitch change was -4.4+/-5.8 degrees (P = .005). ConclusionFor cleft lip and palate patients with maxillary hypoplasia, surgical advancement with RED results in counterclockwise rotation of the maxillary occlusal plane.

PB2395 A TALE OF 9 CITIES: THALASSAEMIA IN GUANGXI PROVINCE, CHINA

Background:At the invitation of two Chinese volunteer organizations, Dandelion and Sunflower Foundations, we visited YuLin, Beilu, Rongxian, Luchuan, Nanning, Guigang, Guiping (Twice), WuZhou and BaiSe during July and November 2018 and their surrounding regions From earlier acquired information development of services for thalassemia patients were in very early stages. Some children with thalassemia major are abandoned by their parents because they cannot afford to pay for the transfusions and the chelation therapy. Many of these children are then adopted to families in various western countries.Aims:The aim was to evaluate the children, suggest how treatment may be improved and where possible provide assistance so that the families may access all necessary treatment modalities so that they do not reach a state of despair and then abandon their child or children.Methods:An update on thalassemia was presented in English and translated to Chinese. Parents, who wished to have their children evaluated, were interviewed and their children were examined. In some centers written information was collected. In addition, some home visits were undertaken in some surrounding rural areas to evaluate a number of children, who had been identified by the Foundations. The information acquired are presented in the results. Clinical evaluations were performed. Verbal consent was obtained for anonymous publication of data and photographs. Because of time restraints and as evaluations had not always been formally arranged 157 patients In addition another 25 were briefly evaluatedResults:Data was recorded on 97 boys and 60 girls. Mutations on 93 were homozygous 17(5), 41‐42/17 (11),41‐42/IVS II 654(16), 41‐42/‐28(19),17/‐28(4),IVSII 654/‐28(1),homozygous 71‐72 (3), 71‐72/‐28 (1), HbE/Thal (2), HbH Constant Springs (12) and w with an SEA alpha mutation. Age was 4months to 14years(Mean 7.9yrs). Pre‐transfusion Hb range was 30‐90 g/L (Mean 66) and ferritin 135 to 13,000ng/ml (n = 120 mean 3700). Many children had significant frontal bossing, maxillary hyperplasia and gum margin hyperplasia. Spleen was often palpable at between 3‐6 cm below costal margin and in one case was 16 cm. Chelation was below recommended doses and very irregular. Deferasirox was taken by 20 (mostly Indian origin), 23 occasional deferroxamine, 24 deferiprone, 17 combination of latter two, 2 on deferasirox and deferiprone and 3 all three chelators. Two patients were receiving Chinese traditional medicine. Two patients were also taking Thalidomide in order to increase their hemoglobinSummary/Conclusion:The presentations seemed to empower the parents. It became clearly accepted that pre‐transfusion Hb levels should be between 90‐100 g/L. Advice on optimal chelation was given in every instance where indicated. Where necessary and feasible, resources for assistance or sponsorship to enable parents to pay for transfusion, chelation, bone marrow transplantation when safe and donor had been identified, were put into action. Even though we try to avoid splenectomy, it was recommended for a number of children. We encouraged the Foundations to extend their activities as much as possible throughout the provence. Overall, even though a relatively small number of patients were seen, this cross section of population with thalassemia reflects the province‐wide status and also some mechanisms by which assistance could be given to maximize outcomes.

The long face syndrome

Since the beginning of cephalometric analysis, a significant number of approaches to vertical facial proportions have been proposed. In our view, diagnosing a vertical problem has nothing to do with numbers or measurements. It's all about perceptions and - rather subjective - impressions. An "impression" of long face may be provoked by the following causes isolated or combined: vertical maxillary hyperplasia, open bite, anterior vertical mandibular hyperplasia, narrow face, lack of chin-neck definition. Clinical planning in these cases is followed by 3D simulation and CAD/CAM generation of surgical splints. Surgical management of the Long Face Syndrome may include the following strategies isolated or combined : maxillary impaction, vertical chin reduction, counterclockwise rotation of the occlusal plane.

Orodental manifestations of facial port-wine stains

Patients with facial port-wine stains (PWS) often demonstrate oral manifestations of their disorder; however, the spectrum and prevalence of such findings among a cohort of patients with PWS has not been established. As a result, dermatologists and oral health specialists may be uncertain how to counsel their patients with PWS regarding oral hypervascularity, bony oral changes, and oral hygiene. We sought to identify physical findings and complications involving the teeth, oral cavity, and perioral structures in individuals with facial PWS. This was a cross-sectional study of 30 patients with facial PWS. Descriptive data were collected through anonymous paired surveys completed by patients and their dentists, and analyzed (Fisher exact test) for trends based on physical findings and stage of the PWS. The most common orodental manifestations according to patients were enlargement of the lip (53.3%), stained gums (46.7%), abnormal bite (30%), and spontaneous bleeding of the gums (26.7%). Staining of the gingiva correlated significantly with gingival hyperplasia (P = .006), maxillary hyperplasia (P = .014), and widened interdental spaces (P = .002), and in all cases gingival staining predated these findings. Lip hyperplasia was reported more frequently by patients than by their dentists (50% vs 18.2%, P = .008). Orodental manifestations were more common among patients with darker and thicker PWS. Hemorrhage after dental procedures was rare (4.5%). Modest sample size and difficulty recruiting control subjects are limitations. Facial PWS commonly affect the orodental structures, and intraoral staining may predict future complications.

Medical and Quality of Life Indications for Splenectomy in Children with Hereditary Spherocytosis.

Abstract 5082 BackgroundSplenectomy has been considered the treatment of choice for patients with “symptomatic” hereditary spherocytosis (HS). However, given the long-term risks associated with asplenia, including sepsis and thrombosis, the risks and benefits of splenectomy in HS require ongoing analysis. To our knowledge, no comprehensive institutional review summarizing the medical and quality of life indications for splenectomy in children with HS has been reported to date. MethodsA retrospective record review was performed of 64 children, age ' 18 yrs, having splenectomy for HS at Children's Medical Center Dallas from January 1973 through March 2009. The following patient characteristics were abstracted: age at splenectomy, family history of HS and/or splenectomy, baseline hemoglobin concentration and reticulocyte count prior to splenectomy, degree of splenomegaly, total number of prior transfusions, hyperhemolytic events, and aplastic crisis. Patients were classified based on mean hemoglobin concentration as having mild (≥11.0 g/dL), moderate (8.0-10.9 g/dL), or severe HS ('7.9 g/dL) (Bolton-Maggs PH, et al. Br J Haematol 2004;126:455-74). The primary indication for splenectomy was characterized in each case as either a “quality of life (QOL) indication” or a “medical indication.” QOL indicators included jaundice, pallor, maxillary hyperplasia, poor growth, fatigue, exercise intolerance, and family history of splenectomy for HS which, having been deemed successful, fostered parental preference for splenectomy in their child. Medical indicators included baseline hemoglobin approaching transfusion-dependent threshold, multiple or severe hyperhemolytic events, severe aplastic crisis, symptomatic gallbladder disease, marked splenomegaly, and the need for numerous transfusions. Some patients had both medical and QOL indications but only one was designated as the primary indication for splenectomy based on documentation in the medical record. ResultsWhen classifying HS patients by hemoglobin concentration, 16 patients (25%) were mild, 38 (59%) moderate, and 10 (16%) severe. The following table summarizes the patient characteristics.Patient CharacteristicsMild HS (n=16)Moderate HS (n=38)Severe HS (n=10)Median age at splenectomy, yr11.410.34.7Family history of HS, n (%)16 (100%)25 (65.8%)6 (60%)Family history of splenectomy, n (%)14 (87.5%)18 (47.4%)5 (50%)Pre-splenectomy: Hemoglobin level (g/dL), median (range)11.6 (11.0-13.7)9.7 (8.2-10.9)7.4 (5.0-7.9)Reticulocyte count (%), median (range)9.8 (3.9-28.7)11.7 (1.1-21.1)12.4 (6.5-25.3)Splenomegaly (cm), median (range)3.5 (0-7.0)4.0 (0-12.0)4.7 (2.0-8.0)Despite having just mild HS, 16 patients had splenectomy, 6 for QOL indications. Moreover, 11 of 38 moderate HS patients had splenectomy for a primary QOL indication. Medical indications predominated in all 10 patients with severe HS. Of the 17 patients with QOL indications who had splenectomy, 8 (47%) also had documentation of medical indications. Among 45 patients with primary medical indications for splenectomy, 18 (40%) also had QOL issues contributing to the decision. Two patients' indications for splenectomy were unclear. ConclusionsAlthough splenectomy is generally felt to be unnecessary in patients with mild HS and many with moderate HS as well, our experience demonstrates that splenectomy is often performed primarily for QOL indications in both risk categories. A validated assessment tool that incorporates QOL indicators in addition to the traditional medical reasons for splenectomy in patients with HS would be of great value in an evidence-based assessment of the patient's need for splenectomy. We recommend inclusion of QOL indicators when considering splenectomy in patients with mild and moderate HS. DisclosuresNo relevant conflicts of interest to declare.

Segmental odontomaxillary dysplasia: An unusual orthodontic challenge

Segmental odontomaxillary dysplasia has only recently been delineated from other osseo-odontogenic dysplasias. Yet, this seldom-reported developmental abnormality might be diagnosed by a unique combination of clinical, radiographic, and histologic findings. Unilateral maxillary hyperplasia combined with displaced molars and unusual bone trabeculation is quite obvious at an early age. Recognition and subsequent reporting by dental specialists is encouraged to more clearly define the natural progression and the appropriate treatment of this abnormality. (Am J Orthod Dentofacial Orthop 2003;123:84-6)

Endoscopic treatment of sinonasal disease in patients who have had orthognathic surgery

Certain skeletofacial patterns may be predisposed to aggravated sinonasal disease postoperatively. These may include, but are not limited to, facial skeletal asymmetries with high septal deviations and those with obstructive nasal respiration and mouth breathing that leads to skeletal growth disturbances such as vertical maxillary hyperplasia and apertognathism. These sinonasal diseases may partly be the result of osteomeatal blockage by pre-existing structures, or synechial shelves and webs blocking normal maxillary antral mucosal flow.The use of nasal antral windows placed anteriorly in the lateral nasal wall at the time of downfracture LeFort (Hosaka window) do not seem to benefit the drainage of the maxillary antrum. This is because physiological flow often bypasses this region.If patients present postoperatively with new sinonasal disease or the aggravation of pre-existing symptoms, evaluation by both endoscopically assisted intranasal and axially and coronal computed tomography (CT) is recommended. Functional endoscopic sinus surgery by the minimally invasive Messerklinger technique, combined with intranasal use of laser-assisted turbinoplasty and soft tissue lysis, have been successfully used for most of these patients.Because the anatomical positioning of the midfacial structure can potentially affect patients with a predisposition to sinonasal physiological disturbances, consideration should be given to preoperative evaluation and discussion of potential consequences.

Condylar remodelling and resorption after Le Fort I and bimaxillary osteotomies in patients with anterior open bite: A clinical and radiological study aesthetic and reconstructive surgery

A sample of 259 patients with vertical maxillary hyperplasia, mandibular hypoplasia and anterior vertical open bite, collected from three different institutions, was analysed regarding temporomandibular joint (TMJ) sounds, condylar remodelling, and condylar resorption. All patients underwent Le Fort I osteotomies, and bilateral sagittal split advancement osteotomies were performed in 117 patients. Intraosseous wire fixation was used in 149 and rigid internal fixation in 110 patients. Cephalometric and orthopantomographic radiographs were available before surgery, immediately after surgery, one year postoperatively and at the latest follow up. The mean follow up was 69 months (range 20–210 months). The number of patients with TMJ sounds decreased from 38% to 31%. At the latest follow up 23.6% of the patients showed condylar remodelling, 7.7% unilateral condylar resorption and 7.7% bilateral condylar resorption. condylar contours, as dssessed on orthopantomographic radiographs, were classified as five different types. Condyles with preexisting radiological signs of osteoarthrosis or having a posterior inclination were at high risk for progressive resorption. Female patients with severe anterior open bite, high mandibular plane angle and a low posterior-to-anterior facial height ratio, who underwent a bimaxillary osteotomy, were prone to condylar resorption. Bone loss was predominantly found at the anterior site of the condyle. The incidence of condylar resorption was significantly higher after bimaxillary osteotomies (23%) than after only Le Fort I intrusion osteotomies (9%). Avoidance of intermaxillary fixation by using rigid internal fixation tended to reduce condylar changes, in particular in patients who underwent only a Le Fort I osteotomy. Rigid internal fixation in bimaxillary osteotomies resulted in condylar remodelling in 30% and progressive condylar resorption in 19% of the patients. Condylar changes were not significantly different after using either miniplate osteosynthesis or positional screws in bilateral sagittal split osteotomy procedures.

Skeletal and dento-alveolar stability of Le Fort I intrusion osteotomies and bimaxillary osteotomies in anterior open bite deformities: A retrospective three-centre study

A sample of 267 patients with maxillary hyperplasia, a Class I or Class II/I occlusion and anterior vertical open bites, collected from three different institutions, was analysed regarding stability after surgical corrections. Skeletal and dento-alveolar stability of the maxilla, and positional changes of the mandible and of the incisors were evaluated. All patients underwent Le Fort I intrusion osteotomies and in 92 patients segmentation of the maxillae was performed. An additional bilateral sagittal split advancement osteotomy was performed in 123 patients. Intraosseous wire fixation was used in 153 patients and rigid internal fixation in 114 patients. Cephalometric radiographs were collected before orthodontic treatment, before surgery, immediately after surgery, one year postoperatively and at the latest follow up. The mean follow up was 69 months (range 20–210 months). It can be concluded that patients with anterior open bites, treated with a Le Fort I osteotomy in one-piece or in multi-segments, with or without bilateral sagittal split osteotomy, exhibited good skeletal stability of the maxilla. Rigid internal fixation produced the best maxillary and mandibular stability. The mean overbite at the longest follow up was 1.24 mm and a lack of overlap between opposing incisors was present in 19%. The overbite did not differ significantly between the different treatment procedures, probably due to compensatory movements of the mandibular and maxillary incisors.

Hemimaxillofacial dysplasia: A report of two new cases and further delineation of the disorder

Miles, Lovas, and Cohen first described hemimaxillofacial dysplasia in two patients in 1987. This disorder consists of facial asymmetry, facial hypertrichosis, unilateral maxillary hyperplasia, and hypoplastic teeth. We report two additional cases with similar findings.