Fwd: Craniofacial Surgery

Surgical Treatment of Polyostotic Craniomaxillofacial Fibrous Dysplasia Associated With Acromegalia: The McCune: –: Albright Syndrome Introduction: The McCune–Albright syndrome (MAS) is a complex congenital disorder caused by the embryonic post-zygotic somatic activating mutations in the GNAS1 gene. In such syndrome, phenotypes are heterogeneous and comprised polyostotic/monostotic fibrous dysplasia, café au lait macules, and hyperfunctioning endocrinopathies as the excess growth hormone. Likewise, acromegaly, as a manifestation of the endocrine hyperfunction, is unusual and affects about 20% of patients with MAS. Case Presentation: This research study describes a case of a 31-year-old female subject presenting polyostotic fibrous dysplasia with severe facial involvement, along with acromegaly and the MAS. The case was satisfactorily managed by surgical re-alignment and presented no clinical signs of relapse in a 12-year follow-up period. Finally, a literature review was conducted to discuss the standard protocols and the controversies when treating such cases. Conclusion: Patients with craniomaxillofacial fibrous dysplasia associated with acromegaly may present significant facial deformities that can be satisfactorily treated by cosmetic treatment, especially in patients with psychological problems and severe social acceptance. Address correspondence and reprint requests to Jéferson Martins Pereira Lucena Franco, DDS, Department of Oral and Maxillofacial Surgery, Hospital Geral de Fortaleza—HGF, Rua Paulo Morais, 321, Papicu, Fortaleza, Ceará, Zip Code: 60175-175, Brazil; E-mail: jefersonlucenaodonto@hotmail.com Received 26 June, 2018 Accepted 21 February, 2019 The authors state that they have no conflict of interest. © 2019 by Mutaz B. Habal, MD.

Cranial Base Superimposition of Cone-Beam Computed Tomography Images: A Voxel-Based Protocol Validation Objectives: The primary objective of the present study was to find the gold-standard accuracy of voxel-based superimposition of cone-beam computed tomography (CBCT) datasets with a protocol developed for the Dolphin Imaging 3D software. The secondary objectives were to analyze reproducibility and efficiency of this protocol. Study Design: Twenty-five CBCT datasets of patients with dental implants present were selected. Each Base Volume dataset was duplicated to create a second volume. Subsequently, both volumes were superimposed with a voxel-based protocol consisting of 3 successive steps "Side-by-side Superimposition"; "Overlay Superimposition"; and "Export Orientation to 2nd Volume". The protocol's accuracy was evaluated by measuring the mean distance between the apex of each dental implant on the Base Volume and second volume datasets. Efficiency was given by the mean time needed to complete all superimposition steps. Reproducibility was analyzed by calculating the intraclass correlation coefficients. Results: Mean time needed to complete the protocol was 198 seconds. The protocol had a rotational accuracy of 0.10° to 019° and a translational accuracy of 0.20 to 0.24 mm. Intra-observer and inter-observer reproducibility were 1 and 0.921 to 1, respectively. Conclusions: The protocol is accurate, precise, reproducible, and efficient. The validation of this method enables unbiased analysis of surgical outcomes based on a single, user-friendly software product that is widely available in academic and clinical settings. Address correspondence and reprint requests to Orion Luiz Haas Junior, DDS, MSc, PhD, Pontifícia Universidade Católica do Rio Grande do Sul, PUCRS, Av. Ipiranga, n.6681, Building 6, Porto Alegre, RS 91530-001, Brazil; E-mail: olhj@hotmail.com Received 31 July, 2018 Accepted 24 February, 2019 Orion Luiz Haas Junior and Raquel Guijarro-Martínez Both co-authors contributed equally to this work. All patients provided written informed consent for the use of their CBCT scans and the Teknon Medical Center ethical committee approved the study (CIR-ECL-2012-03). Brazilian Ministry of Education, Coordination for the Improvement of Higher Education Personnel (Coordenação de Aperfeiçoamento de Pessoal de Nível Superior, CAPES), Programa de Doutorado Sanduíche no Exterior (PDSE), grant no. 99999.006660/2015-00 awarded to Orion Luiz Haas Junior. The authors report no conflicts of interest. © 2019 by Mutaz B. Habal, MD.

Intra- and Postoperative Blood Loss and Transfusion Requirements in Children Undergoing Craniofacial Surgery Pediatric craniosynostosis (CS) surgery is frequently associated with extensive blood loss and transfusion requirements. The aim of the study was to evaluate the authors' institutional procedure with 2-surgeon approach and early transfusion strategy on blood loss and blood product transfusions in children undergoing craniofacial surgery. A retrospective analysis of medical records was performed of pediatric CS corrections during a 15-year period. Primary endpoint was blood loss and transfusion requirement during and the following 24 hours postoperatively. Linear regression analyses were performed of associations between intra and- postoperative blood loss and blood loss and weight. A total of 276 children (median 9 months) were included. Intraoperative blood loss was 22 mL/kg (14–33 mL/kg) and postoperatively 27 mL/kg (18–37 mL/kg), with no change during the study period. Intraoperative transfusions of red blood cell and plasma were 16 mL/kg (10–24 mL/kg) and postoperative 14 mL/kg (9–21 mL/kg). Postoperative red blood cell and plasma transfusions were 2 mL/kg (0–6 mL/kg) and of 0 mL/kg, respectively. Craniosynostosis type was related to blood loss (P < 0.001). There was an association between intraoperative and postoperative blood loss (P = 0.012) and intra- and postoperative blood loss and weight (P = 0.002, P = < 0.001). Duration of surgery was 110 minutes (range 60–300 minutes). Pediatric CS surgery is associated with substantial intra- and postoperative blood loss and transfusion requirements, which did not change over a 15-year period. Blood loss was associated with type of CS. Intraoperative blood loss was correlated to postoperative blood loss and body weight. Address correspondence and reprint requests to Alexander Lindholm D'Amore, MD, Department of Anaesthesiology, Aarhus University Hospital, Palle Juul-Jensens Boulevard, DK-8200 Aarhus N, Denmark; E-mail: alexanderdamore@gmail.com Received 3 October, 2018 Accepted 18 February, 2019 Source of funding: The study from Novo Nordisk Fonden (NNF14OC0011787). The authors report no conflicts of interest. © 2019 by Mutaz B. Habal, MD.

Comprehensive Treatment of Facial Deformity Due to Radiotherapy in Rhabdomyosarcoma Patients: Distraction Osteogenesis and Free Flaps Surgical Technique Radiotherapy is essential for achieving and maintaining local control in head and neck rhabdomyosarcoma patients. However, radiotherapy may cause outgrowth disturbances of facial bone and soft tissue, resulting in facial asymmetry. Several studies have shown that the management of irradiated bones still remains challenging. The possibility of obtaining functional and aesthetic results when managing facial deformities due to radiation therapy with distraction osteogenesis combined with free flaps reconstruction is not common and not well documented in recent literature. In this report, we present the long-term results of distraction osteogenesis and soft tissue reconstruction via microvascular free flap to correct the facial hypoplasia of a young patient who underwent radiation therapy for rhabdomyosarcoma. This is the first presentation of 26 years long-term results in a patient who also underwent free flaps reconstruction as ancillary surgery for esthetic good results. Address correspondence and reprint requests to Marco Cicciù, DDS, PhD, Department of Biomedical and Dental Sciences and Morphological and Functional Imaging, Messina University, School of Dentistry University of Messina, Policlinico G. Martino, Via Consolare Valeria, Messina, 98100, ME, Italy; E-mail: acromarco@yahoo.it Received 5 November, 2018 Accepted 18 December, 2018 The authors report no conflicts of interest. © 2019 by Mutaz B. Habal, MD.

Evaluation of the Facial Soft-Tissue Thickness in Patients With Cleft Lip and Palate Aim: In this study the authors aimed to evaluate craniofacial morphology and facial soft tissue thickness of the patients with cleft lip and palate.(CLP), and to compare the results with a sex and age-matched noncleft healthy control group. Methods: Craniofacial and soft tissue thickness measurements of 20 patients with unilateral cleft lip palate (UCLP) and 20 patients with bilateral cleft lip palate (BCLP) were compared with 20 noncleft control subjects. Angles between Sella-Nasion-A point, Sella-Nasion-B point, Nasion-A and Nasion-B line, Sella-Nasion line and Gonion-Gnation line, and linear (glabella, rhinion, subnasale, labrale superius, stomion, labrale inferius, labiomentale, pogonion, and gnathion) measurements were assessed on lateral cephalometric radiographs. Group differences were evaluated with 1-way analysis of variance and post hoc Tukey test. Results: Unilateral cleft lip palate and BCLP groups demonstrated lower values of skeletal measurements than control group except for Sella-Nasion line and Gonion-Gnation line value (P < 0.01). Maxillary incisors were retruded in BCLP group compared with the other groups (P < 0.001). Thickness of the rhinion, subnasale, and stomion demonstrated no significant difference between UCLP and BCLP groups (P > 0.05). Thickness of rhinion and stomion was found significantly higher in both groups, and thickness of the subnasale was found significantly lower than the healthy group (P < 0.05). Conclusion: Findings of the study suggested that patients with CLP showed differences on craniofacial morphology and facial soft tissue thickness. Before planning orthodontic treatment and esthetic surgeries, soft tissue thickness should be taken into consideration in addition to skeletal measurements in CLP patients. Address correspondence and reprint requests to Emire Aybuke Erdur, PhD, Emire Aybuke Erdur Department of Orthodontics, Faculty of Dentistry, Necmettin Erbakan University, 42050 Konya, Turkey; E-mail: dtaybuke@gmail.com Received 24 October, 2018 Accepted 24 February, 2019 The authors report no conflicts of interest. © 2019 by Mutaz B. Habal, MD.

"Three Subunit" Classification of Lesser-Form Cleft Lip and Muscle Analysis and Reconstruction of These Subunits Based on the Microanatomic Structures of Muscles in the Normal Upper Lip Background: The main phenotypic features of lesser-form cleft lip include nasal, philtrum, and vermilion deformities. The manifestations of lesser-form cleft lip vary greatly, and it is difficult to rebuild these subunits during the operation. Methods: "Three subunits" classification of lesser-form cleft lip was identified as nasal deformity (N), philtrum deformity (P), or vermilion deformity (V); and slight deformity (0) or obvious deformity (1). A total of 200 patients with lesser-form cleft lip were classified into one of the following 8 types: N1P1V1, N1P1V0, N0P1V1, N1P0V1, N0P0V1, N1P0V0, N0P1V0, or N0P0V0. Then the authors discussed the deformities of the lesser-form cleft lip and the reconstruction of the muscles in these subunits based on the microanatomic structure of the nasolabial muscle. Results: This retrospective review included 200 patients with a lesser-form cleft lip, who were seen at our center from 2015 to 2017. There were 149 (74.5%) N1P1V1, 11 (5.5%) N1P1V0, 13 (6.5%) N0P1V1, 4 (2.0%) N1P0V1, 8 (4.0%) N0P0V1, 10 (5.0%) N1P0V0, 1 (0.5%) N0P1V0, and 4 (2%) N0P0V0 clefts. The various deformities of nasal floor, philtrum ridge, and vermilion may suggest that the muscle bundles in these subunits are abnormal and the operation should be performed to simulate the running directions and tension lines of these local muscles. Conclusions: The microanatomic structure and the tension lines of the nasolabial muscles can provide new perspectives for better understanding and repairing the lesser-form cleft lip in subunits. Address correspondence and reprint requests to Tao Song, MD, Department of Cleft Lip and Palate, Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100144, PR China; E-mail address: songtao2059@163.com Received 21 November, 2018 Accepted 15 February, 2019 The authors report no conflicts of interest. © 2019 by Mutaz B. Habal, MD.

The Right to Heresy: Letters to the Editor No abstract available

Statistical Shape Analysis of Cerebellum in Patients With Chiari Malformation I The surgical procedure is viewed as a typical treatment choice for patients with Chiari malformation (CM). Decompression is the preferred method for surgery, but it is not always possible to understand whether decompression has been successful especially in an early period. The present study focuses on investigating the shape differences in the cerebellums of Chiari patients compared with healthy controls, and to assess the clinical application of this situation whether if present. The MRI scans were reviewed retrospectively. Cerebellar data were obtained from the digital images and 9 anthropometric landmarks were marked on each image. Shape difference was assessed by performing Generalized Procrustes analysis. The cerebellar shape deformation from control to the patient was evaluated performing the Thin Plate Spline approach. There is a statistically significant cerebellar shape difference between groups. Highest deformation was determined at the cerebellar tonsillar inferior area, posterior of the uvula, and anterior of inferior medullary velum. The present study demonstrated cerebellar shape differences in CM I patients using a landmark-based geometric morphometric approach, considering the topographic distribution of cerebellum for the first time. Address correspondence and reprint requests to Gokhan Ocakoglu, PhD, Uludag University Medical School, Department of Biostatistics, 16059 Bursa, Turkey; E-mail: gocakoglu@gmail.com Received 4 December, 2018 Accepted 13 February, 2019 The authors report no conflicts of interest. © 2019 by Mutaz B. Habal, MD.

A Craniometric Analysis of the Posterior Cranial Base After Posterior Vault Distraction Background: Posterior vault distraction osteogenesis (PVDO) has been demonstrated to effectively increase intracranial volume, treat increased intracranial pressure, and improve head shape in syndromic patients. The purpose of this study is to compare changes along the posterior cranial base before and after distraction. Methods: A retrospective review was completed of subjects who underwent PVDO with computed tomography scans at 2 time-points: within 3 months preoperatively and 1 to 6 months postoperatively. Using Mimics software, craniometric landmarks were identified and surface area of the foramen magnum was calculated. A comparison of pre- to postoperative measurements was completed using Wilcoxon matched-paired signed rank tests and linear regression. Results: A total of 65 PVDO subjects were identified, 12 subjects met inclusion criteria. Mean operative age was 3.0 ± 4.0 years. The cranial vault was distracted on average 25.0 ± 6.0 mm, with those < 12 months of age distracted 29.5 ± 4.9 mm and >12 months of age distracted 22.0 ± 4.9 mm (P = 0.0543). There was a significant increase in pre- to postoperative foramen magnum surface area (52.1 ± 63.2 mm2, P = 0.002), length (0.9 ± 1.4 mm, P = 0.050), and width (0.6 ± 1.0 mm, P = 0.050). Similarly, linear distances between nasion and posterior cranial base landmarks such as foramen magnum (3.4 ± 4.2 mm, P = 0.010), and occipital protuberance (9.1 ± 9.6 mm, P = 0.003) were increased. Subjects under 12 months had a greater percentage increases in posterior vault length than those over 12 months. Conclusion: Posterior vault distraction osteogenesis is associated with an increase in size of the foramen magnum, and lengthening of the posterior cranial base, both of which may be beneficial in patients with turribrachycephaly. Address correspondence and reprint requests to Jesse A. Taylor, MD, Division of Plastic and Reconstructive Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Colket Translational Research Building, 3501 Civic Center Blvd, 9th floor, Philadelphia, PA 19104; E-mail: taylorj5@email.chop.edu Received 8 February, 2018 Accepted 20 February, 2019 The authors report no conflicts of interest. Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (www.jcraniofacialsurgery.com). © 2019 by Mutaz B. Habal, MD.

Comparison Between Intermaxillary Fixation With Screws and an Arch Bar for Mandibular Fracture Purpose: Intermaxillary fixation (IMF) using the arch bar has been the standard technique for a long time in the management of mandibular fracture. But because of several complications, intermaxillary fixation with screws has been introduced and adopted to use. This investigation compared the outcomes and complications between the traditional IMF and modified intermaxillary fixation with screws techniques in terms of the malocclusion, surgical time, root injury, and screw or arch bar failure. Methods: This retrospective review included 66 patients who underwent reconstruction of mandibular fractures that uses traditional arch bar or IMF screws as IMF. Preoperative and postoperative facial bone computed tomography, panoramic x-ray were used to evaluate the patients. The complaints and complications were recorded in electric medical record based on patients' consultation during follow-up. Results: The total complication rate was no higher using anchoring screw as an IMF than using arch bar fixation as an IMF. Mean surgical time was significantly shorter in the anchoring screw group than arch bar fixation (111 versus 157 min; P < .05). After compensation of the number of fracture sites, there was a significant difference in operation time (linear regression model, P = .009). Conclusion: This investigation revealed that the IMF using IMF screw systems could be a good alternative for the management of mandibular fracture. It could minimize the whole operation time with the similar complication ratio. Address correspondence and reprint requests to Jong-Woo Choi, MD, PhD, Department of Plastic and Reconstructive Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea; E-mail: pschoi@amc.seoul.kr Received 4 March, 2018 Accepted 14 February, 2019 The authors report no conflicts of interest. © 2019 by Mutaz B. Habal, MD.