Surgical Management of Hypertelorism: The 3D Animated Technical Note.

The hypertelorism surgery is a complex procedure requiring a long learning curve. Even though the box osteotomy technique is well described in literature, its representation is generally based on texts and illustrations that do not really give a 3-dimensional or a dynamic point of views. The authors present a 3-dimensional animated video, Supplemental Digital Content 1, http://links.lww.com/SCS/E561 showing the craniofacial osteotomies and focusing on the critical points to correct hypertelorism.

Computer-Assisted Surgery in Facial Bipartition Surgery.

PURPOSE: Frontonasal dysplasia or median cleft syndrome is a rare condition often associated with transverse maxillary deficiency for which facial bipartition is the proper surgical treatment. This technical note describes the use and efficacy of 3-dimensional virtual surgical planning and patient-specific cutting guides in facial bipartition. MATERIALS AND METHODS: An 11-year-old boy presented with frontonasal dysplasia and a midline facial cleft. RESULTS: Computed tomographic (CT) scan visualized an intercanthal distance of 40 mm and a maxillary width at the second molar level of 46.2 mm. The osteotomies, amount of bone removal, and movement of the osteotomized bone were planned virtually based on CT findings: the intended postoperative intercanthal distance and maxillary width were 25.5 and 49.6 mm, respectively. Customized cutting guides and titanium miniplates were manufactured. Postoperative CT scan showed an intercanthal distance of 25.5 mm and a maxillary width of 49.7 mm. CONCLUSION: The intercanthal distances and maxillary widths were similar between the simulation and postoperative CT images, confirming the accuracy and utility of computer-assisted surgery in facial bipartition.

Orbital Lipomatosis: A Complication of Steroid Therapy in the Sweet Syndrome.

The description of a Sweet syndrome steroid dependant-induced orbital lipomatosis is reported. A 76-year-old-man with history of Sweet syndrome presented with severe bilateral proptosis (Hertel value, 25 mm) with decreased visual acuity and evoked potentials lengthened. A bilateral transpalpebral orbital decompression was performed by resection of intraorbital fat without bone removal. The surgery was uneventful. The volume of resected orbital fat was 15 ml for both sides. Proptosis reduction was 6 mm. Postoperative Hertel values were 19 mm, and evoked potentials were improved. The proptosis was managed successfully. Orbital lipectomy led to minimal sequelae and may be repeated if necessary in this case.

Long-Term Assessment of Suturectomy in Trigonocephaly and Anterior Plagiocephaly.

INTRODUCTION: Techniques for treating trigonocephaly and anterior plagiocephaly have evolved from the initial suturectomy technique to frontal cranioplasty. The authors evaluated the suturectomy technique in adolescent patients with these craniosynostoses, by carrying out a retrospective, long-term assessment at the end of growth. METHODS: Patients with anterior plagiocephaly or trigonocephaly were included. All had undergone coronal or metopic suturectomy with bilateral orbitofrontal bandeau resection between 1997 and 2005. The primary endpoint was the Whitaker classification. The secondary endpoints were anthropometric measurement, assessments of the bone defects on computed tomography scan, and the comments of patients and their relatives concerning the final skull outcome. Patients with anterior plagiocephaly also attended an ophthalmological consultation. RESULTS: Seventeen patients were included in the study: 8 with anterior plagiocephaly and 9 with trigonocephaly. Mean age at the time of surgery was 6.91 months. Mean age at the time of craniofacial consultation was 14 years. Fourteen patients (82%) were classified as Whitaker Class III and IV, corresponding to poor esthetic results and persistent bone defects. Seven patients requested further surgery. CONCLUSION: This study shows that suturectomy seems to yield poor esthetic results in the long term and patients should be followed up throughout adolescence to correct any craniofacial deformities.

Frontofacial Monobloc Advancement With Simultaneous Frontal Cranioplasty in Adolescents With Residual Apert Syndrome Deformations.

INTRODUCTION: The treatment of faciocraniosynostosis has steadily evolved since the introduction of craniofacial surgery in the 1950s. The aim of this study is to demonstrate the positive results obtained by frontofacial monobloc advancement with simultaneous frontal cranioplasty in adolescents with adult facial bones and residual Apert syndrome deformations. MATERIALS AND METHODS: Three adolescents underwent surgery between September 1, 2010 and March 31, 2011. All had faciocraniosynostosis in the context of Apert syndrome and had undergone brain decompression surgery during the first year of life. However, they presented intracranial hypertension. The authors carried out frontofacial monobloc advancement with internal distraction and frontal cranioplasty. RESULTS: The mean frontal advancement was 13.8 mm. The mean maxillary advancement was 16.3 mm. About exorbitism, 2 patients had grade III and 1 had grade I before surgery. After monobloc advancement, 2 patients had no exorbitism and 1 had grade I. About dental occlusion, 3 patients had class III before surgery and were overcorrected in class II after advancement. DISCUSSION: Frontofacial monobloc advancement yields satisfactory functional and esthetic results in these cases. In conclusion, simultaneous frontofacial monobloc advancement and cranioplasty appears to be a promising technique for the treatment of adolescents with residual craniofacial deformations.

Reconstruction of the orbitozygomatic framework: State of the art and perspectives.

The reconstruction of the whole orbitozygomatic framework (OZF) is complex and can be encountered in cases of congenital midface deformity, after tumor ablative surgery and in severe facial trauma. Nowadays, surgeon has a wide range of available techniques that have continually grown over the past years, optimizing the surgical management and the aesthetical outcomes. Among them, the autologous bone graft (ABG) remains one of the most suitable options : ABG is easy to harvest and has optimal biological properties for bone healing. It can be tailored to the patient anatomy thanks to the recent advances in computer-assisted surgery. However, substantial drawbacks remain such as the early resorption of the non-vascularized graft, the need of a donor site and its potential morbidity. Alloplastic reconstruction is another option that can resolve both the resorption issue and the donor site morbidity. Moreover, the 3D-printing technologies also allows the manufacturing of patient specific implants. However, alloplastic materials have a variable success, especially due to the high risk of infection or exposure. Consequently, regenerative medicine is a promising field that aims to find a procedure without the disadvantages of ABG or alloplastic based reconstructions, but displaying similar or even higher success rate. Indeed, recent tissue engineering strategies have demonstrated encouraging results for bone regeneration using natural or synthetic biomaterials, patient cells and synthetic bioactive substances. The objective of this review is to present the etiologies of OZF defect, the available reconstruction procedures as well as the current state of the research.

Facial dog bites in children: A public health problem highlighted by COVID-19 lockdown.

BACKGROUND: Stay-at-home injunction during COVID-19 pandemic led to new dynamics in households and increased the risk of domestic accidents involving pets. The aim of the study was to demonstrate an increase of facial dog bites in children during first lockdown period in France, compared to the same period in 2018 and 2019. Secondary objective was to investigate the demographics and circumstances in which dog bites occurred. METHODS: A retrospective multicentric study was conducted nationwide. Patients under 18 years old managed in fifteen oral and maxillofacial surgery departments for a dog bite were included. RESULTS: Eighty-seven patients were included. A significant increase of the number of children managed for facial dog bite was noticed in 2020 (p=0.0005). The male-to-female ratio was significantly reversed in 2020 with more bites in girls than boys (p=0.02). In 2020, children were mostly bitten to cheeks (28.6 %), lips-and-chin region (26.2 %), and eyelids (23.8 %). Severe bites increased in 2020, in comparison with 2018 and 2019. Dog bites occurring while petting or playing significantly increased in 2020 (31 %) (p=0.03). CONCLUSION: The process leading to bites is highly dependent on the balance of dog-owner relationship. This was strongly disrupted during COVID-19 pandemic, resulting in the increase of dog bites in households. Regarding dog bites, face is the most vulnerable area in children. Its injury has lots of esthetic and functional consequences and maxillofacial surgeons have a key role to play in their prevention. Reminders of some of these management and prevention strategies are presented in this article.

Tongue reconstruction after oncological resection: Analysis of the functional outcomes.

The curative management of oral squamous cell carcinoma can be responsible for swallowing and/or speech impairments. In this study, we analyzed the functional outcomes in patients who underwent an oncological resection and a primary reconstruction of the tongue and/or of the floor of the mouth (TFM). We also investigated the predictive factors for poor functional outcomes. This retrospective study included operated patients from October 2013 to May 2021 at the TOURS University Hospital. We assessed the functional results two years after the completion of the cancer treatment with self-administered questionnaires quantifying swallowing and speech disorders. Thirty-three patients were included and reconstructed with antebrachial free flap (N = 16), local flap (N = 8) or Biodesign ® membrane (N = 9). A higher proportion of pT1 tumor was observed in patient who had a Biodesign-based reconstruction (p = 0.001). There was no significant difference between the groups in terms of postoperative complications or for the duration of enteral feeding. The 21 patients who had an adjuvant radiation therapy had no significant more altered functions. Functional scores were significantly higher in the free flap reconstruction group (DHI =24 and SHI=21) (p = 0.008). No predictive factors for poor outcomes were observed. The repair of TFM defects must be adapted to the resection size. The reconstruction techniques allow to get acceptable functional outcomes even for the greater tumors or in case of radiation therapy. Further research would be required to better identify the predictive factors for poor outcomes.

Reconstruction of large defects of the lips and commissure using a composite radial forearm palmaris longus free flap associated with a lengthening temporalis myoplasty.

We performed a single-stage operation to reconstruct a large defect of the lips and commissure using a composite radial forearm-palmaris longus free flap. To obtain cranial traction and a voluntary smile, independently from any jaw movement, traction was achieved by using a lengthening temporalis myoplasty. The tendon attached to the coronoid process was fixed to the palmaris longus tendon, recreating a new commissure and a "neo-modiolus." Physical therapy was started on the 21st postoperative day to facilitate progress from a "mandibular smile," to ideally a spontaneous and symmetric smile after 3 months of therapy. This procedure was able to obtain good oral continence and a good commissural movement during smile which has not previously been mentioned in the published literature.

Late reactivation of cherubism in an adult further to local inflammation.

Cherubism is a rare pediatric disease affecting the jaw. It appears among children between 2 and 5 years old. Maximum growth is observed at 7-8 years old, then lesions remain unchanged or increase slowly until puberty. Only 2 cases of later growth have been reported. We describe a case of cherubism reactivation in a 46-year-old woman. Appearance of a new lesion occurs in a context of local inflammation due to repeated friction of the dental prosthesis on the mandible. No article in the literature describes a similar case. This case shows the determining role of inflammation (local or general) in the pathophysiology of cherubism.

Computer-Assisted Frontofacial Monobloc Advancement and Facial Bipartition for Pfeiffer Syndrome: Surgical Technique.

BACKGROUND: In patients with Pfeiffer syndrome, several corrections are required to correct facial retrusion, maxillary deficiency, or even hypertelorism. The frontofacial monobloc advancement (FFMA) and the facial bipartition (FB) are the gold standard surgeries. We present the correction of this deformity using a simultaneous computer-assisted FFMA and FB. METHODS: The 3-dimensional surgical planning defined the virtual correction and bone-cutting guide in view of the FFMA and FB. Coronal and intraoral approaches were combined to perform the osteotomies. Four internal distractors were also placed for the postoperative distraction osteogenesis. RESULTS: We reported 2 cases of computer-assisted surgery with satisfying outcomes. The sagittal deficiency (fronto-facial retrusion) was corrected by FFMA and the transversal abnormality (i.e., hypertelorism and maxillary deficiency) by the FB, then followed by an internal distraction osteogenesis. CONCLUSIONS: Computer-assisted surgery is helpful and a reliable option for the management of complex faciocraniosynostosis such as hypertelorism and frontofacial retrusion.

Management of post enucleation socket syndrome patients - A retrospective study.

The aim of this retrospective monocentric study was to report the outcomes following the correction of post enucleation socket syndrome (PESS) as well as the factors predicting for a multistep management. Patients were included, if they had received surgery to manage PESS. Demographics and patient history were collected. The symptoms of PESS were clinically rated by the same surgeon and ocularist, preoperatively and after each procedure. The surgery was considered as successful when no PESS clinical symptoms were observed. A prognostic study was performed to investigate the surgical outcomes. RESULTS: Thirty-six patients were included and eight of them had their PESS corrected after the first surgery. In the multivariate analysis (MVA), the deep upper lid sulcus symptom remained the main significant factor associated with an incomplete correction after the first surgery (OR 45.5, IC 95% (3.481-594.6), p = 0.004). For further corrections, the ptosis was the only significant prognostic factor requiring several surgeries (p = 0.005). At the end of the management, 94.4% of the patients had satisfying outcomes. CONCLUSIONS: The management of a PESS involves the correction of both the orbital cavity and the soft tissues. Although its correction is complex and time consuming in the majority of cases, the patients should be informed that the final cosmetic outcomes remain good at the expense of several surgeries.

Sentinel lymph node biopsy versus selective neck dissection in patients with early oral squamous cell carcinoma: A cost analysis.

OBJECTIVE: Sentinel lymph node biopsy (SLNB) has been proved to be as efficient as selective neck dissection (SND) for the treatment of occult metastases in T1-T2cN0 oral squamous cell carcinoma (OSCC). The aim of our study was to assess and compare the cost of these two surgical procedures. PATIENTS AND METHODS: This retrospective cost analysis includes consecutive patients treated between 2012 and 2017 in two French hospitals either by SLNB or SND. Hospital cost (hospital stay for initial surgery and re-hospitalizations over a period of 60 days after the initial surgery), the length of hospital stay for the initial surgery and the perioperative management were described and compared between the two techniques. The propensity score regression adjustment method was used to address selection bias. RESULTS: Ninety-four patients underwent SLNB procedure and seventy-seven patients underwent SND. The length of hospital stay for initial surgery was lower in SLNB group: 5.8 days (SD: 3.8) versus 9.2 days (SD: 5) in the SND group. Hospital costs were lower in SLNB group: €7 489 (standard deviation: €3 691) versus €8 886 (standard deviation: €4 381) but this difference was not significant after propensity score regression adjustment. The rate of complication, the delay of full oral feeding and postoperative drainage were lower in SLNB group. CONCLUSION: SLNB in T1-T2cN0 OSCC is less invasive than SND with fewer complications, a shorter length of hospital stay and favorable perioperative management. This study shows that this technique could be also less expensive than SND.

Standardized and axially vascularized calcium phosphate-based implants for segmental mandibular defects: A promising proof of concept.

The reconstruction of massive segmental mandibular bone defects (SMDs) remains challenging even today; the current gold standard in human clinics being vascularized bone transplantation (VBT). As alternative to this onerous approach, bone tissue engineering strategies have been widely investigated. However, they displayed limited clinical success, particularly in failing to address the essential problem of quick vascularization of the implant. Although routinely used in clinics, the insertion of intrinsic vascularization in bioengineered constructs for the rapid formation of a feeding angiosome remains uncommon. In a clinically relevant model (sheep), a custom calcium phosphate-based bioceramic soaked with autologous bone marrow and perfused by an arteriovenous loop was tested to regenerate a massive SMD and was compared to VBT (clinical standard). Animals did not support well the VBT treatment, and the study was aborted 2 weeks after surgery due to ethical and animal welfare considerations. SMD regeneration was successful with the custom vascularized bone construct. Implants were well osseointegrated and vascularized after only 3 months of implantation and totally entrapped in lamellar bone after 12 months; a healthy yellow bone marrow filled the remaining space. STATEMENT OF SIGNIFICANCE: Regenerative medicine struggles with the generation of large functional bone volume. Among them segmental mandibular defects are particularly challenging to restore. The standard of care, based on bone free flaps, still displays ethical and technical drawbacks (e.g., donor site morbidity). Modern engineering technologies (e.g., 3D printing, digital chain) were combined to relevant surgical techniques to provide a pre-clinical proof of concept, investigating for the benefits of such a strategy in bone-related regenerative field. Results proved that a synthetic-biologics-free approach is able to regenerate a critical size segmental mandibular defect of 15 cm3 in a relevant preclinical model, mimicking real life scenarii of segmental mandibular defect, with a full physiological regeneration of the defect after 12 months.

Mechanical characterization and optoelectronic measurement of parietal bone thickness before and after monocortical bone graft harvest: design and validation of a test protocol.

Parietal bone grafts are commonly used in craniomaxillofacial surgery. However, bone removal may weaken the parietal bone and lead to deleterious complications. The aim of our study was to design a test protocol for characterization of the impact resistance of parietal bone before and after monocortical bone graft harvest and to validate an optoelectronic measurement of parietal bone thickness. Twelve fresh human cadaver heads were used for the validation study. To evaluate impact resistance, we developed a pendulum Charpy impact testing machine. The impact force was gradually increased until failure (fracture) of the defined parietal bone area. According to the protocol, we measured the maximum absorbable energy or impact force to failure. With our test setup, measurement of the impact resistance of parietal bone was accurate to within 0.025 J. We defined a range of values and particularly a threshold value. The initial maximal impact must not to exceed 4 J. For more accuracy, we compared 5 nondestructive measurement methods using a surgical navigation system with optoelectronic tracking. We achieved an algorithm based on 2 methods that ensured a measurement resolution of 0.1 mm. Validation of this protocol will allow us to evaluate the loss of strength resulting from bone removal and the correlation between strength and thickness of the parietal bone.

Disability in a medieval village community: A unique case of facial dysmorphism.

OBJECTIVE: To identify the pathology causing the severe facial dysmorphia of a medieval individual from the site of Rigny (Indre-et-Loire, France) and to evaluate its functional repercussions on the subject's hearing and social life. MATERIALS: An individual from Rigny for the osteological study, 69 individuals from the region for the Ct-Scan study and 48 individuals from the site for the isotopic analysis. METHODS: Macroscopic analysis was performed using standard osteological methods. Consequences of pathology on hearing were assessed by CT-scan. Dietary behaviour was analysed by stable carbon and nitrogen isotope analysis of bone collagen. RESULTS: The individual is a woman who was over 50 years of age at the time of her death with a severe form of Treacher-Collins syndrome that resulted in deafness. No osteological signs of maltreatment were observed and there is no evidence that this individual's diet was different from that of the rest of the community. CONCLUSIONS: All information testifies to the integration of this individual into the village population. SIGNIFICANCE: This study is the first archaeological description of Treacher Collins syndrome. It shows the ability of the paleopathological approach to help identify the attitudes of societies for which written sources are most often lacking. LIMITATIONS: In the absence of palaeogenomic analysis it is not possible to identify the origin of this case. SUGGESTIONS FOR FURTHER RESEARCH: Increase the paleopathological semiology by CT-scan in order to specify the consequences of pathologies and integrate isotopic analyses to enrich discussion about perceptions of disease.

Absence of inferior labial and lingual frenula in Ehlers-Danlos syndrome: a minor diagnostic criterion in French patients.

BACKGROUND: Ehlers-Danlos syndrome (EDS) is a heterogenous group of diseases that can be potentially life threatening in the vascular form. The diagnosis is mainly based on a clinical score including many items that demonstrate skin, mucosa, joint, and vessel involvement. The score is particularly helpful in hypermobile and classical forms but can be normal in the vascular type. The absence of the lingual and inferior labial frenula was reported to be a useful diagnostic tool in a series of 12 patients with classical and hypermobile EDS. However, two further reports have contested the value of this sign. OBJECTIVE: To determine the diagnostic value of the absence of inferior labial or lingual frenula in EDS. METHODS: Patients with EDS were prospectively recruited from November 2006 to April 2007 in a French tertiary center. Each patient was examined to check for the presence or absence of the oral frenula and matched with two controls seen from February to May 2007. RESULTS: Forty-three patients (ratio female/male of 2.07 : 1, mean age 31 years, range 4-63 years), 4 with classical EDS, 19 with hypermobile EDS, and 20 with vascular-type EDS, were included and matched with 86 controls. The sensitivity of the absence of the inferior labial frenulum was 42% (95% CI 27, 58) and for the lingual frenulum was 53.5% (95% CI 38, 69). The specificity was 99% (95% CI 94, 100) and 98% (95% CI 92, 100), respectively. In the vascular group, 13 of 20 patients were affected (sensitivity = 65% [95% CI 41, 85]; specificity = 97% [95% CI 87, 100]; odds ratio = 72 [95% CI 8, 645]). Inter-observer agreement was excellent (kappa value 0.91). CONCLUSION: The absence of the inferior labial frenulum or lingual frenulum is a specific sign of EDS that can be easily checked by a physician. It can be helpful in the early diagnosis of the disease, including the vascular type, in affected families. It can also be helpful to distinguish between joint hypermobility syndrome and the hypermobile type of EDS in patients with joint hypermobility.

Ultrastructural demonstration of a relationship between acquired cutis laxa and monoclonal gammopathy.

Acquired cutis laxa is an uncommon disorder sometimes associated with monoclonal gammopathy and multiple myeloma, although the mechanism of this link is unclear. We report here a case of a 34-year-old man with generalized acquired cutis laxa and monoclonal light chain disease with renal and neurological involvement. Electron microscopy examination of a skin sample revealed shortened and fragmented elastic fibres in the reticular dermis and normal collagen bundles. Immunogold labelling revealed anti-lambda antibodies closely bound to the microfibrillar component of elastic fibres, thus supporting a causal relationship between monoclonal gammopathy and the changes in skin elasticity.

How Accurate Is Computer-Assisted Orbital Hypertelorism Surgery? Comparison of the Three-Dimensional Surgical Planning with the Postoperative Outcomes.

Importance: Surgery of orbital hypertelorism (ORH) remains an imprecise surgical procedure depending on the experience and habits of the craniofacial surgical teams. Computer-assisted surgery (CAS) has developed dramatically in craniofacial surgery, but there is no current study assessing its accuracy for ORH surgery. Objective: This study aimed to assess the input of CAS and especially the accuracy of the cutting guide-based procedures. The authors presented the computer-assisted box osteotomy or facial bipartition techniques and compared the preoperative surgical planning with the postoperative results. Design, Setting, and Participants: A monocentric retrospective study included the patients who underwent surgical correction of ORH from 2016 to 2019 at the University Hospital Center of Tours, France. All the patients had a computer-assisted orbital hypertelorism surgery (CAOHS) using cutting guides and tailored fixation plates. Of 10 patients included, 7 were treated by box osteotomies and 3 by facial bipartitions. Intervention: Each patient had a preoperative computed tomography (CT) scan enabling a virtual simulation of the reconstruction and the manufacturing of patient-specific cutting guides and customized osteosynthesis plates. The postoperative CT scans were compared with the three-dimensional (3D) virtual simulation using the distances between the sagittal plane and orbital and infraorbital reference points, and from the measurement of the orbital advancement (i.e., 10 reference measurements). Results: All patients had satisfactory clinical and aesthetical outcomes with a mean interorbital distance of 22.8 ± 2.8 mm. The postoperative measurements were significantly higher than for the surgical planning (p < 0.0001). The average absolute differences between the 3D virtual planning and the postoperative CT scans were <1.30, 1.90, and 0.80 mm for the orbital, infraorbital, and orbital advancement measurements, respectively. The overall accuracy of the CAOHS (root mean square deviation) was 1.39 mm. Conclusions: The use of computer-assisted design and computer-aided manufacturing device, such as cutting guides and tailored plates, facilitates the bony surgical correction of ORH using box osteotomy or facial bipartition and allows for valuable, reproducible, and satisfactory clinical outcomes.