Comparison of closed percutaneous screw reduction versus open reduction and internal fixation in the treatment of frontal sinus fractures: A retrospective study.

The aim of this study was to compare closed percutaneous screw reduction to traditional open reduction-internal fixation (OR-IF) for the treatment of anterior table fractures. Both groups were evaluated in terms of operative variables, complications, and treatment success. Of 32 patients included, 19 patients underwent OR-IF, while 13 underwent percutaneous screw reduction. The median operative time, length of hospital stays, and treatment cost of the OR-IF group were 100 min (range 60-130), 4 days (range 3-9), and $727 (range $642-$1291), respectively. The same variables for the closed reduction group were 30 min (range 20-40), 2 days (range 1-2), and $303 (range $252-$349), respectively. The closed reduction group exhibited a shorter operative time (p< 0.001), reduced length of hospital stays (p< 0.001), lower treatment cost (p< 0.001), and a lower complication rate (p = 0.025) compared to the OR-IF group. Late-term outcomes in both groups showed no visible contour changes or step deformities. In conclusion, the percutaneous screw reduction technique is a safe and effective option with minimal morbidity in the treatment of frontal sinus anterior table fractures. Therefore, traditional OR-IF should be reserved for fractures that are not suitable for reduction using minimally invasive techniques.

Biallelic truncating variants in VGLL2 cause syngnathia in humans.

BACKGROUND: Syngnathia is an ultrarare craniofacial malformation characterised by an inability to open the mouth due to congenital fusion of the upper and lower jaws. The genetic causes of isolated bony syngnathia are unknown. METHODS: We used whole exome and Sanger sequencing and microsatellite analysis in six patients (from four families) presenting with syngnathia. We used CRISPR/Cas9 genome editing to generate vgll2a and vgll4l germline mutant zebrafish, and performed craniofacial cartilage analysis in homozygous mutants. RESULTS: We identified homozygous truncating variants in vestigial-like family member 2 (VGLL2) in all six patients. Two alleles were identified: one in families of Turkish origin and the other in families of Moroccan origin, suggesting a founder effect for each. A shared haplotype was confirmed for the Turkish patients. The VGLL family of genes encode cofactors of TEAD transcriptional regulators. Vgll2 is regionally expressed in the pharyngeal arches of model vertebrate embryos, and morpholino-based knockdown of vgll2a in zebrafish has been reported to cause defects in development of pharyngeal arch cartilages. However, we did not observe craniofacial anomalies in vgll2a or vgll4l homozygous mutant zebrafish nor in fish with double knockout of vgll2a and vgll4l. In Vgll2 -/- mice, which are known to present a skeletal muscle phenotype, we did not identify defects of the craniofacial skeleton. CONCLUSION: Our results suggest that although loss of VGLL2 leads to a striking jaw phenotype in humans, other vertebrates may have the capacity to compensate for its absence during craniofacial development.

A Clinical Report of the Complete Nasal Agenesis: Reconstruction of Congenital Arhinia and Review of the Literature.

Congenital absence of the nose or arhinia is an exceptionally rare craniofacial malformation, and the pathophysiology of the arhinia is still unknown. Most arhinia patients can have difficulties with breathing and feeding due to the absence of the nose, nasal cavities, and associated problems. A 38-day-old female patient was referred to our clinic with arhinia. Physical examination revealed the complete agenesis of nasal structures as the nasal bones and vestibulum nasi. The region of the absent nose was flat and firm at palpation. Congenital arhinia may occur with other associated malformations such as ocular, ear, palate, and gonadal. Therefore, it is recommended to evaluate computed tomography/magnetic resonance imaging in the postnatal period. Additionally, a radiological evaluation will help nasal reconstruction by documenting changes in nasal and maxillary anatomy over time. Due to the limited number of arhinia cases presented, the surgical management of this condition has not been standardized. We presented the pyramid-shaped cartilage grafts for the nasal framework and an expanded paramedian forehead flap for the skin coverage for reconstruction of arhinia.

Algorithms for the management of frontal sinus fractures: A retrospective study.

The aim of this study was to evaluate the long-term results and to evaluate the validity of the treatment algorithm that can be safely applied in dfrontal sinus fractures. All patients were evaluated in terms of late-term contour deformity and further craniometric analysis for measurement of maximum amount of displacement, fracture surface area (mm2) and the maximum angulation of the fracture (degree) were done. 125 patients (101 male, 24 female) with frontal sinus fractures with an average age of 22.4 years (range,17-66 years) were reviewed. All patients with isolated anterior table fractures without displacement were followed up on conservative basis. 33 patients with anterior table fractures with displacement and 39 patients with anterior and posterior table fractures were also followed on conservative basis without surgical intervention. The cut-off value of the maximum amount of displacement was confirmed to be 4.5 mm in prediction of late-term contour deformities (p < 0.001). The maximum amount of displacement was decreased by an average of 1.8 mm at late-term. Apart from the standard protocols, within the limitations of the study it seems that isolated anterior table fractures with a maximum amount of displacement of less than 4.5 mm can be treated conservatively without leading to contour deformities. CSF leakage in the acute setting might not always require cranialization and this may spontaneously resolve within 10 days. Cranialization should be considered whenever CSF leakage lasts longer than 10 days.

The Relevance of Fluid and Blood Management Using Microcirculatory Parameters in Children Undergoing Craniofacial Surgery.

ABSTRACT: Perioperative management of bleeding in children can be challenging. Microvascular imaging techniques have allowed evaluating the effect of blood transfusion on the microcirculation, but little is known about these effects in children. We aimed to investigate the effects of blood management using macro- and micro-hemodynamic parameters measurement in children undergoing craniofacial surgery. This is a prospective observational repeated measurement study including fourteen children. The indications for blood transfusion were changes of hemoglobin/hematocrit (Hct) levels, the presence of signs of altered tissue perfusion and impaired microcirculation images. Total and perfused vessel densities, proportion of perfused vessels, microvascular flow index, and systemic parameters (hemoglobin, Hct, lactate, mixed venous oxygen saturation, K+, heart rate, mean arterial blood pressure) were evaluated baseline (T1), at the end of the surgical bleeding (T2) and end of the operation (T3). Four patients did not need a blood transfusion. In the other 10 patients who received a blood transfusion, capillary perfusion was higher at T3 (13[9-16]) when compared with the values of at T2 (11[8-12]) (P < 0.05) but only 6 patients reached their baseline values. Although blood transfusions increased Hct values (17 ±â€Š2.4 [T2]-19 ±â€Š2.8 [T3]) (P < 0.05), there was no correlation between microvascular changes and systemic hemodynamic parameters (P > 0.05). The sublingual microcirculation could change by blood transfusion but there was not any correlation between microcirculation changes, hemodynamic, and tissue perfusion parameters even with Hct values. The indication, guidance, and timing of fluid and blood therapy may be assessed by bedside microvascular analysis in combination with standard hemodynamic and biochemical monitoring for intraoperative bleeding in children.

Presentation of Infantile Hemangiopericytoma/Solitary Fibrous Tumor as a Giant Extracranial Temporal Mass.

BACKGROUND: Infantile Hemangiopericytoma (HPC)/Solitary Fibrous Tumor (SFT), a vascular tumor of head and neck region, can be congenital or arise during the first year of the life. As the infantile form of hemangiopericytoma has a better course than the adult form, surgical excision is recommended. Case Report: A full-term neonate presented with a congenital right temporal soft tissue mass. MRI revealed a highly vascular mass with a hemorrhagic and possible necrotic core without intracranial extension. The lesion grew in 2 weeks from 4x4 cm to 9x7 cm. Histologically, a hypercellular spindle cell mesenchymal neoplasm had prominent staghorn vessels, alternating with hypocellular areas. Mitotic activity was low(1-3/HPF) and necrosis was absent. Conclusion: Infantile HPC/SFT of head and neck can grow rapidly during the infantile period. Complete excision without mutilating surgery should be curative.

Thirty Years Later: What Has Craniofacial Distraction Osteogenesis Surgery Replaced?

LEARNING OBJECTIVES: After studying this article and viewing the video, the participant should be able to: 1. Compare the relative stability and neurosensory changes following mandible distraction osteogenesis with those after traditional advancement and fixation. 2. Describe the condylar changes that can occur after mandible distraction osteogenesis and list three ways to mitigate these changes. 3. Propose clinical situations where segmental or rotational movements of the midface may allow improved outcomes compared to en bloc linear distraction advancement. 4. Summarize the advantages and risks associated with anterior and posterior cranial distraction osteogenesis compared to traditional one-stage expansion. SUMMARY: Over the past 30 years, distraction forces have been applied to the spectrum of craniofacial osteotomies. It is now time to assess critically the current understanding of distraction in craniofacial surgery, identifying both traditional procedures it has replaced and those it has not. This article provides a review of comparative studies and expert opinion on the current state of craniofacial distraction compared with traditional operations. Through this critical evaluation, the reader will be able to identify when distraction techniques are appropriate, when traditional techniques are more favorable, and what the future of distraction osteogenesis is.

A Novel Method of Neo-osseous Flap Prefabrication: Induction of Free Calvarial Periosteum with Bioactive Glass.

BACKGROUND: Reconstruction of craniofacial bone defects is a primary focus of craniofacial surgery. Although autogenous bone grafts remain as the gold standard, alloplastic materials have also gained widespread popularity due to their off-the-shelf availability, ease of use, and durability. In addition to replacing the missing bone, some of these alloplastic materials have also been found to induce new bone formation. OBJECTIVES: In this study, the phenomenon of neo-osseous induction with bioactive glass was investigated for different implant-soft tissue configurations. MATERIALS AND METHODS: Thirty-two male, Wistar albino rats were divided into four equally numbered study groups. In group 1 (FP), adipofascial groin flaps were prefabricated with free periosteal grafts. In group 2 (FPB), adipofascial groin flaps were prefabricated with free periosteal grafts and bioactive glass. In group 3 (FB), adipofascial groin flaps were prefabricated with bioactive glass. In group 4 (control), adipofascial groin flaps were not prefabricated. Morphometric analyses of the prefabricated structures were performed using micro-CT. The histologic properties of the ectopic ossification were assessed by using a modified scoring system. RESULTS: Group 1 (FP) showed the greatest rate of mature lamellar bone formation. Group 2 (FBP) showed the greatest amount of bone density and volume. However, the addition of bioactive glass in group 2 (FBP) decreased the rate of mature lamellar bone formation when compared with group 1 (FP). Ectopic ossification was not observed in the control group. CONCLUSION: Bioactive glass can be successfully used in the prefabrication of vascularized compound structures for the reconstruction of complex bone defects. However, interference with the periosteal induction of mature lamellar bone formation should be taken into consideration, especially in pediatric bone defects, which primarily rely on spontaneous osteogenesis through periosteal induction.

Distraction of Fronto-Orbital Segment as a Nonvascularized Bone Graft in Craniosynostotic Patients.

Fronto-orbital advancement using distraction techniques involves the dura left attached to the osteotomized bone segment to avoid dead space formation and dural injury, whereas it is impossible to reshape the supraorbital bar and the frontal bone complex, when necessary. Our approach combines advantageous parts of conventional and distraction osteogenesis techniques as remodel and distract the supraorbital bar and frontal bone complex as a free bone graft. Twenty-seven patients either being syndromic and nonsyndromic craniosynostosis, with at least 3 years of follow-up were reviewed in this study. Mean age of the patients at the time of the operation was 23.44 ±â€Š18.42 months and mean operative time was 4.96 ±â€Š0.97 hours. Blood transfusion was required in all patients, with an average of 112.04 ±â€Š44.60 mL. Amount of the distraction ranged 10 to 30 mm, a mean of 17.26 ±â€Š4.71 mm for the right side and 18.15 ±â€Š4.69 mm for the left side. Mean duration of consolidation was 98.26 ±â€Š12.98 days and mean follow-up was 41.33 ±â€Š22.92 months. In this study, result of internal distraction of fronto-orbital segment as a nonvascularized bone graft in craniosynostotic patients is reviewed to emphasize the efficacy of the nonvascularized bone graft distraction in management of craniosynostosis. Graft distraction after fronto-orbital and cranial vault remodeling appears to be safe and effective approach in correcting severe craniosynostosis deformities especially necessitating asymmetrical advancement.

Microvascular Tissue Transfers for Midfacial and Anterior Cranial Base Reconstruction.

Reconstruction of a midfacial defect can represent a difficult challenge for the plastic surgeon. Although many midfacial deformities have traumatic or congenital origins, the vast majority of head and neck defects occur after resection of malignant head and neck neoplasms. Autogenous reconstruction is now routinely performed for larger, complex defects resulting from surgical resection or trauma. In this study, the authors present 27 patients with midfacial defects reconstructed with free flaps. Twenty-two of the defects were created by surgical ablation of cancer (maxillectomy) and the others were traumatic. The maxillectomy defects were classified into 4 according to the classification proposed by Cordeiro. Eighteen of the patients were male and 9 were female. Twenty-nine free flaps were performed. Six different types of flaps including radial forearm flap, vertical rectus abdominis (VRAM) flap, anterolateral thigh (ALT) flap, tensor fasciae latae (TFL) flap, fibula osteocutaneous flap, and iliac osteocutaneous flap were accomplished. Types I and II defects were reconstructed with radial forearm flap. Type III defects were reconstructed with VRAM and ALT. Type IV defects were reconstructed with VRAM and TFL. Two patients underwent a second flap reconstruction due to recurrent disease (9.1%). Average patient age was 53.1 years. Free-flap survival was 100%. Free tissue transfer is the method of choice in midfacial reconstruction. Following a reconstructive algorithm is useful in the decision-making process for patient evaluation and treatment. Every reconstructive microsurgeon might have different experiences with different flaps. Therefore, the algorithm for flap choices is not universal among surgeons.

Long-term stability of surgical-orthodontic treatment for skeletal Class III malocclusion with mild asymmetry.

Skeletal Class III malocclusion with mandibular prognathism is often associated with mandibular asymmetry, and patients with Cl III deformity and asymmetry may undergo orthognathic surgery to improve facial function and esthetics. However, the long-term stability of mandibular setback surgery has been considered "problematic". We report a 25-year-old female patient who was diagnosed as having a skeletal Class III relationship, mandibular asymmetry and unilateral crossbite. She underwent bilateral sagittal split-ramus osteotomy and orthodontic therapy, and this led to improvement of her dentofacial relationship, esthetics and occlusion. The outcome was stable after 8 years of retention. This case shows that asymmetry correction using mandibular setback surgery for skeletal Class III deformity can yield long-term stability.

De novo mutations in SMCHD1 cause Bosma arhinia microphthalmia syndrome and abrogate nasal development.

Bosma arhinia microphthalmia syndrome (BAMS) is an extremely rare and striking condition characterized by complete absence of the nose with or without ocular defects. We report here that missense mutations in the epigenetic regulator SMCHD1 mapping to the extended ATPase domain of the encoded protein cause BAMS in all 14 cases studied. All mutations were de novo where parental DNA was available. Biochemical tests and in vivo assays in Xenopus laevis embryos suggest that these mutations may behave as gain-of-function alleles. This finding is in contrast to the loss-of-function mutations in SMCHD1 that have been associated with facioscapulohumeral muscular dystrophy (FSHD) type 2. Our results establish SMCHD1 as a key player in nasal development and provide biochemical insight into its enzymatic function that may be exploited for development of therapeutics for FSHD.

Dermal fibroblast transcriptome indicates contribution of WNT signaling pathways in the pathogenesis of Apert syndrome.

Çetinkaya A, Taskiran E, Soyer T, Simsek-Kiper PÖ, Utine GE, Tunçbilek G, Boduroglu K, Alikasifoglu M. Dermal fibroblast transcriptome indicates contribution of WNT signaling pathways in the pathogenesis of Apert syndrome. Turk J Pediatr 2017; 59: 619-624. Cranial sutures are unossified connective tissue structures between the cranial bones, which allow expansion of these bones during development. Premature ossification of these structures is called craniosynostosis. Apert syndrome is a well-defined genetic syndrome, which is characterized by craniosynostosis and arises as a result of two missense mutations in Fibroblast Growth Factor Receptor, type 2 gene (FGFR2). In this study, differentially expressed genes in dermal fibroblasts from individuals with Apert syndrome and controls were investigated to identify important pathways in the pathogenesis of Apert syndrome. For this purpose, primary skin fibroblast cultures obtained from 3 individuals with Apert syndrome and 3 controls without craniosynostosis were compared by transcriptome microarray, GeneChip Human Genome U133 Plus 2.0. As a result, 181 genes were shown to be differentially expressed between experimental groups. Among these, 10 genes, which significantly differ in Apert syndrome fibroblasts compared to controls, were shown to be involved in a common interaction network and have common Gene ontology (GO) biological processes terms. COL11A1, COMP, CPXM2, ITGA8, MGF and TNC are differentially expressed genes that have GO terms associated with extracellular matrix (ECM) organization, while FRZB, SFRP2 and WNT2 are involved in WNT signaling pathway. Reorganization of ECM and changes in WNT signaling pathway show that Apert syndrome primary fibroblast cultures may have an increased potential for bone differentiation. The results of this study support craniosynostosis in Apert syndrome may be the result of fast and early differentiation of connective tissue along the sutures.

An extremely rare complication following frontoorbital advancement: infarction of the recurrent artery of Heubner.

Many intracranial as well as extracranial complications can be seen following craniosynostosis surgeries. In this article, we would like to share an extremely rare complication of the infarction of the recurrent artery of Heubner, occurred following frontoorbital advancement. In this case, an 18-month-old male patient underwent supraorbital bar and frontal bone remodeling surgery for nonsyndromic metopic suture synostosis. The preoperative neurosurgical evaluation revealed no signs of increased intracranial pressure. On the 3rd postoperative day, the patient developed asymmetric smile and weakness on the left extremities. Computerized tomography showed a hypodense infarction region around the right basal ganglia and internal capsule, concordant with the region supplied by the recurrent artery of Heubner. The patient's symptoms started to regress on the 2nd day of enoxaparine treatment and he was discharged on 12th postoperative day with almost no signs of the event. In this paper, we presented an unlikely complication after frontoorbital advancement. Keeping in mind the long operating time and the proximity of the procedure to the central nervous system, assessment of the neurological function of the patients both before and after the operation and rapid intervention in case of development of neurologic symptoms are of great importance.

Functional outcomes of mandibular distraction for the relief of severe airway obstruction and feeding difficulties in neonates with Pierre Robin sequence.

The purpose of this study was to review the application of mandibular distraction to relieve severe airway obstruction or feeding problems of neonates. Thirteen neonates with Pierre Robin sequence who underwent bilateral mandibular distraction between 2010 and 2013 for relief of their severe airway obstruction or feeding problems were retrospectively reviewed. The mean preoperative and postoperative airway diameters were 3.89±1.64 and 9.03±1.98 mm. respectively and significant difference was observed with distraction (p < 0.001). The rate of severe airway infection also significantly decreased from 69.2% to 23.1% (p=0.016). 84.6% of the patients were able to be fed orally at discharge whereas 6 patients (46.2%) required support via orogastric tube before distraction (p=0.125). No growth disturbance, dental complications or malocclusion was observed in the long-term follow up. Mandibular distraction appears to be a promising and effective surgical option for relieving airway obstruction and feeding problems in severe Pierre Robin Sequence patients.

Congenital maxillomandibular syngnathia: a new management technique using distraction techniques.

Complex zygomaticomandibular syngnathia is an extremely rare condition with an unknown etiology. The main goal of the surgery is to release the ankylosis, establish good functioning mandible, and prevent reankylosis, if possible. In our case, we offer a new solution to have an adequate oral opening and to prevent reankylosis. After the release of bony syngnathia, we placed a distractor between mandibular segment and maxillozygomatic complex. To our best knowledge, this is the only syngnathia case in the literature treated using distraction techniques. There is a major improvement in the patient's status. Distraction may broaden our horizons in this rare and difficult-to-treat deformity.

Aggressive giant cystic lymphatic malformation in a newborn.

Lymphatic malformations are uncommon, benign and congenital malformations of the lymphatic system exhibiting lack of development of communication between the lymphatic and venous circulation. We report the unusual case of rapidly expanding giant lymphatic malformation of the torso, bilateral axillae and left upper extremity of a newborn. As the first-line treatment, aspiration and sclerotherapy with bleomycin were performed. The sclerotherapy failed to cause regression of the mass, and rapid expansion of the malformation necessitated surgery. Partial resection of the mass was performed. Clinical symptoms of respiratory distress resolved in the early postoperative period, and the patient became hemodynamically stable. However, intrathoracic invasion of the mass developed, leading to restriction of thoracic expansion, ending in death. In conclusion, surgical treatment of giant lymphatic malformations remains challenging.

Spontaneous lateral temporal encephalocele.

A spontaneous encephalocele is one that develops either because of embryological maldevelopment or from a poorly understood postnatal process that permits brain herniation to occur. We here report a rare case of lateral temporal encephalocele extending to the infratemporal fossa under the zygomatic arch. At birth, the infant was noted to have a large cystic mass in the right side of the face. After being operated on initially in another center in the newborn period, the patient was referred to our clinic with a diagnosis of temporal encephalocele. He was 6 months old at the time of admission. Computerized tomography scan and magnetic resonance imaging studies revealed a 8 × 9 cm fluid-filled, multiloculated cystic mass at the right infratemporal fossa. No intracranial pathology or connection is seen. The patient was operated on to reduce the distortion effect of the growing mass. The histopathological examination of the sac revealed well-differentiated mature glial tissue stained with glial fibrillary acid protein. This rare clinical presentation of encephaloceles should be taken into consideration during the evaluation of the lateral facial masses in the infancy period, and possible intracranial connection should be ruled out before surgery to avoid complications.