Percutaneous Sclerotherapy of Large Venous Malformations Using Consecutive Polidocanol and Bleomycin Foam: MR Imaging Volumetric and Quality-of-Life Assessment.

PURPOSE: To retrospectively evaluate sclerotherapy using consecutive polidocanol and bleomycin foam (CPBF) for large untreated venous malformations (VMs) and/or those resistant to prior treatment. MATERIALS AND METHODS: This retrospective study included all patients treated with CPBF for untreated VMs larger than 10 mL and/or refractory to treatment between May 2016 and October 2019. Baseline and follow-up VM volumes were measured on fat-suppressed T2-weighted magnetic resonance (MR) imaging. Outcome was evaluated on postprocedural MR imaging volumetry and by a retrospective survey assessing clinical response and adverse events. Imaging response was considered good for volume reduction from 50% to 70% and excellent for volume reduction ≥70%. Symptoms and quality-of-life (QoL) scores were compared before and after CPBF sclerotherapy. RESULTS: Forty-five patients (mean age, 16 years; range, 1-63 years; 25 males) with 57 VMs were analyzed and treated by 80 sclerotherapy. Sixty percent (27 of 45) of patients had undergone prior treatment for VM. Median VM volume was 36.7 mL (interquartile range, 84 mL) on pretherapy MR imaging. Good and excellent results after the last sclerotherapy were achieved in 36% (16 of 45) and 29% (13 of 45) of patients, respectively, corresponding to a decrease of >50% in 60% (34 of 57) of VMs. QoL score increased by at least 3 points, regardless of initial symptoms. Most patients did not desire additional sclerotherapy owing to near complete symptomatic relief, even for patients who did not achieve a good response. Swelling, pain, and motor impairment scores significantly improved after CPBF. Adverse events included fever (44%, 15 of 34) and nausea/vomiting (29%, 10 of 34). CONCLUSIONS: CPBF sclerotherapy represents an effective therapy for large and/or refractory VMs with minimal adverse events.

Role of ultra-high-frequency ultrasound in the diagnosis and management of basal cell carcinoma: pilot study based on 117 cases.

BACKGROUND: Ultrasound imaging has recently benefited from the introduction of a new 70 MHz transducer able to provide high-resolution images, i.e. ultra-high-frequency ultrasound (UHFUS). AIM: To study the morphological features of basal cell carcinomas (BCCs) and measure BCC thickness by means of UHFUS examination. METHODS: In this retrospective multicentric study, 171 consecutive patients underwent UHFUS examination between November 2018 and May 2019 for suspected BCC. Diagnosis was confirmed by histopathology. A series of morphological parameters including echogenicity, structure, borders, shape composition (presence of intralesional structures) were investigated along with objective measurements such as thickness (maximum distance between the surface of the epidermis and the deepest part of the tumour) and width. RESULTS: In total, 117 BCCs from 93 patients were examined, including superficial (n = 13; 11.1%), nodular (n = 64; 54.7%), infiltrative (n = 18; 15.4%), mixed subtypes (n = 20; 17.1%) and other subtypes (n = 2; 1.7%). The most frequently observed UHFUS parameters included: hypoechoic signal (n = 80; 68.4%, P < 0.001), homogeneous structure (n = 76, 65.0%, P = 0.01), well-defined borders (n = 77, 65.8%, P < 0.001) and elongated shape (n = 71, 60.7%, P < 0.001). An excellent correlation was found between the BCC thickness measured by UHFUS and the value estimated by histology (interclass correlation ≥ 0.80). CONCLUSION: UHFUS is a new rapid and easy noninvasive skin imaging technique able to provide data on the dimensions and morphology of BCCs in real time and at the bedside. These characteristics mean UHFUS has a number of possible applications, ranging from presurgical mapping to the detection of disease recurrence and treatment monitoring.

Coronal and lambdoid suture evolution following total vault remodeling for scaphocephaly.

OBJECTIVE: Different types of surgical procedures are utilized to treat craniosynostosis. In most procedures, the fused suture is removed. There are only a few reports on the evolution of sutures after surgical correction of craniosynostosis. To date, no published study describes neosuture formation after total cranial vault remodeling. The objective of this study was to understand the evolution of the cranial bones in the area of coronal and lambdoid sutures that were removed for complete vault remodeling in patients with sagittal craniosynostosis. In particular, the investigation aimed to confirm the possibility of neosuture formation. METHODS: CT images of the skulls of children who underwent operations for scaphocephaly at the Hôpital Femme Mère Enfant, Lyon University Hospital, Lyon, France, from 2004 to 2014 were retrospectively reviewed. Inclusion criteria were diagnosis of isolated sagittal synostosis, age between 4 and 18 months at surgery, and availability of reliable postoperative CT images obtained at a minimum of 1 year after surgical correction. Twenty-six boys and 11 girls were included, with a mean age at surgery of 231.6 days (range 126-449 days). The mean interval between total vault reconstruction and CT scanning was 5.3 years (range 1.1-12.2 years). RESULTS: Despite the removal of both the coronal and lambdoid sutures, neosutures were detected on the 3D reconstructions. All combinations of neosuture formation were seen: visible lambdoid and coronal neosutures (n = 20); visible lambdoid neosutures with frontoparietal bony fusion (n = 12); frontoparietal and parietooccipital bony fusion (n = 3); and visible coronal neosutures with parietooccipital bony fusion (n = 2). CONCLUSIONS: This is the first study to report the postoperative skull response after the removal of normal patent sutures following total vault remodeling in patients with isolated sagittal synostosis. The reappearance of a neosuture is rather common, but its incidence depends on the type of suture. The outcome of the suture differs with the incidence of neosuture formation between these transverse sutures. This might imply genetic and functional differences among cranial sutures, which still have to be elucidated.

Craniofacial bone alterations in patients with neurofibromatosis type 1.

Osseous manifestations of neurofibromatosis 1 (NF-1) occur in a minority of the affected subjects but may be because of significant clinical impairment. Typically, they involve the long bones, commonly the tibia and the fibula, the vertebrae, and the sphenoid wing. The pathogenesis of NF-1 focal osseous lesions and its possible relationships with other osseous NF-1 anomalies leading to short stature are still unknown, though it is likely that they depend on a common mechanism acting in a specific subgroup of NF-1 patients. Indeed, NF-1 gene product, neurofibromin, is expressed in all the cells that participate to bone growth: osteoblasts, osteoclasts, chondrocytes, fibroblasts, and vascular endothelial cells. Absent or low content of neurofibromin may be responsible for the osseous manifestations associated to NF-1. Among the focal NF-1 osseous anomalies, the agenesis of the sphenoid wing is of a particular interest to the neurosurgeon because of its progressive course that can be counteracted only by a surgical intervention. The sphenoid wing agenesis is regarded as a dysplasia, which is a primary bone pathology. However, its clinical progression is related to a variety of causes, commonly the development of an intraorbital plexiform neurofibroma or the extracranial protrusion of temporal lobe parenchyma and its coverings. Thus, the cranial bone defect resulting by the primary bone dysplasia is progressively accentuated by the orbit remodeling caused by the necessity of accommodating the mass effect exerted by the growing tumor or the progression of the herniated intracranial content. The aim of this paper is to review the neurosurgical and craniofacial surgical modalities to prevent the further progression of the disease by "reconstructing" the normal relationship of the orbit and the skull.

Control of metopic emissary veins in trigonocephaly surgery. Technical note.

INTRODUCTION: Trigonocephaly with its premature fusion of the metopic synostosis is associated with a risk of cerebral compression and several craniofacial morphological deformations. Numerous surgical techniques have been proposed to enlarge and reshape the forehead. They all carry a risk of bleeding during osteotomies, especially in the region of the superior sagittal sinus (SSS) encased in the early fused suture and of the paired metopic transosseous emissary veins superior to the glabella, which is typical of this type of synostosis. In fact, these paired metopic transosseous veins are often, if not always, the source of major bleeding when torn during the elevation of the frontal flap. TECHNICAL NOTE: A simple technical variant may prevent or at least easily control bleeding in this region during the early phases of the surgical repair. The technical variant to the standard surgical techniques utilized to correct trigonocephaly consists in preserving a triangle of bone above the glabella (about 4 cm at the base and 4 cm in height). The triangle of bone contains the initial segment of the SSS and the emissary metopic veins draining the frontal poles. After removing the frontal flap in a conventional manner, this technical variant allows to detach the veins as well as the SSS from the surrounding bone structures under direct visualization, also in case of vessels running partially encased in the bone. Once the venous structure is detached and hemostasis controlled, this last piece of frontal bone may be removed without unnecessary "iatrogenic" bleeding. CONCLUSION: The propounded procedure does not prolong the surgical time significantly and does not require additional surgical skills or equipment.

Orbital Reconstruction by Patient-Specific Implant Printed in Porous Titanium: A Retrospective Case Series of 12 Patients.

PURPOSE: The purpose of this study was to evaluate the orbital patient-specific implant (PSI) directly printed in porous titanium for the reconstruction of complex orbital bone defects in a series of 12 patients. PATIENTS AND METHODS: The authors designed and implemented a case series. The sample consisted of patients with unilateral complex orbital bone loss. All patients received a porous titanium PSI designed from the healthy contralateral side (mirroring). The criteria analyzed were the functional results: correction of enophthalmos, correction of ocular motility, operative time, complications, and operative revisions. The study was performed from 2015 through 2017. RESULTS: The sample was composed of 12 patients (mean age, 47 yr; age range, 13 to 70 yr). Patients were followed for a mean of 36 weeks postoperatively (range, 4 to 100 weeks). Twelve of the 12 patients presented preoperative enophthalmia, and 8 of the 12 patients presented preoperative diplopia. The mean operating time was 71 minutes (range, 60 to 200 minutes). For 8 patients, the follow-up was simple. In contrast, 2 patients required surgical revision with repositioning of the implant because of intraoperative implant malpositioning with esthetic or functional disturbance and malpositioning was confirmed on the postoperative computed tomogram, 1 patient required explantation of his implant 7 months after the surgery because of spheno-orbital meningioma recurrence (the implant was well positioned), and 1 patient operated on by a subciliary approach presented a postoperative ectropion. In this series of porous titanium orbital PSIs without positioning guides, 17% had malpositioning (2 patients who required a new intervention for repositioning). CONCLUSION: The results of this study suggest that porous titanium PSI could be a surgical option for patients with complex orbital bone defects. In this series 17% of the sample needed a second operation. There are several ways to improve these results, such as intraoperative navigation or integrated positioning guides.

Orbital Cavernous Hemangioma Causing Spontaneous Compressive Hemorrhage.

Cavernous hemangiomas are the most common tumors of the posterior cone of the orbit in adults. They are discovered in a fortuitous manner or in front of the appearance of a progressive exophthalmia.The authors report through a complete literature review (PubMed) patients of retro-orbital cavernous hemangioma revealed by spontaneous compressional hemorrhage from 1984 to 2017.This review is illustrated by a case of sharp decrease in visual acuity secondary to orbital cavernous hemangioma hemorrhage.The authors detail the management and the evolution of all these patients.

Giant Hemifacial Fibrous Dysplasia Functional Treatment and Place of Pamidronate.

The authors report the case of a 9-year-old child with severe form of hemifacial fibrous dysplasia. The authors review the pathology of this treatment modality through the case description and detail the place of pamidronate in the treatment of fibrous dysplasia.

Superior eyelid crease approach for transobital neuroendoscopic surgery of the anterior cranial fossa.

Access to the anterior cranial fossa has traditionally required a large exposure of the surgical field, which can be a source of aesthetic and neurologic morbidity. Minimally invasive surgery approaches have been developed to overcome these adverse effects and was, for a long time, represented by endonasal endoscopic surgery. The superior eyelid crease approach of the anterior cranial fossa with supraorbital osteotomy was described in the early 80s as an interesting alternative to the endonasal approach. This keyhole endoscopic approach allows safe and efficient surgery of the orbit, the anterior cranial fossa, and the mesiotemporal lobe. We successfully treated 10 patients with benign tumors of the anterior cranial fossa, with excellent cosmetic results and no complications. This study presents the surgical technique and its potential indication and limits throughout our experience as well as a review of the literature.

Orthognathic surgery in patients treated with bisphosphonates: A case series.

The aim of this case series was to complete the literature with six other cases of orthognathic surgery in patients treated with bisphosphonate (BP). The majority of studies concerning oral and maxillofacial surgery focus on dental extractions and implant placement in patients treated with BP. Only 5 cases of orthognathic surgery in patients treated with BP have been reported: four cases for osteogenesis imperfecta and one case for osteoporosis. Six patients were included in the study from January 2009 to December 2020: 3 treated for fibrous dysplasia and 3 treated for osteoporosis. BP treatment was stopped for 2 cases because it was the scheduled end of their treatment for fibrous dyplasia. The other four cases didn't stop taking BP for surgery. No precautions for the prevention of MRONJ were taken. The operative follow-up was uneventful. Mucosal healing was satisfactory for all patients at D15. No material exposure were observed. Bone consolidation appears complete on X-rays and on CT-scans at D45 with disappearance and bone filling of the fracture line associated with the absence of painful mobility of the maxilla and mandible on clinical examination. Until now, all the patients have a stable class I occlusion, no signs of osteonecrosis on X-rays (no signs of bone demineralization, no hypermineralized bone sequestration, no signs of osteolysis) or periodontal disease and healed osteotomies of the jaw without radiological features. No relapse were observed. The management of patients treated with bisphosphonate should be done carefully but orthognathic surgery is no longer a contraindication.

Disabling hearing loss after Lefort I osteotomies in patients with history of Eustachian tube dysfunction: An unknown complication.

INTRODUCTION: The aim of this study was to determine the predisposing factors of this deafness after orthognathic surgery for a population with no history of cleft palate. MATERIAL AND METHODS: All patients with significant auditory symptoms after orthognathic surgery performed in the department of Craniomaxillofacial surgery in the Croix Rousse Hospital, Lyon, France, since 2012 were included in the study. The exclusion criterion was a history of cleft palate. Bone movements performed during orthognathic surgery were analyzed. An audiometric assessment, including Pure Tone Audiometry and tympanometry, was performed at day 30 (D30), day 60 (D60) and day 90 (D90). RESULTS: Six patients were included in the study. All patients were women with a mean age of 33.5 years and suffered from class III skeletal deformities. The average hearing loss was 40 dB at D30, 20 dB at D60 and 5 dB at D90 with sometimes differences between the two ears. Five patients had a type C tympanogram suggesting Eustachian tube dysfunction and one patient had a type B tympanogram. All patients benefited from maxillary advancement of 5 mm on average. DISCUSSION: This study allows to conclude that female patients with a history of allergy or catarrhal tubal deafness are at risk of deafness after orthognathic surgery. These patients must be informed of this complication before surgery and be reassured about its reversibility.

Management of intra-osseous arteriovenous malformations of the mandible.

Intra-mandibular arteriovenous malformations (IM-AVM) are rare congenital lesions sadly known by the appearance of cataclysmic haemorrhages. It represents a therapeutic challenge which requires a multidisciplinary approach. This paper aims to evaluate the characteristics and management of IM-AVM through the illustration of six case reports, the largest serie in the literature to our knowledge. This retrospective study included all patients with IM-AVM treated in the hospital of Lyon and Saint-Etienne between 1982 and 2021, in France. The management of mandibular AVMs should be individualized and permanently adjusted according to their stage of development, which makes their management difficult. Furthermore, long-term follow-up is essential to detect and manage the subclinical progression of the lesion. Based on these results and a systematic review of the literature, an algorithm has been developed to guide the treatment strategy for this are pathology.

Stimulation of dental implant osseointegration by low-Intensity pulsed ultrasound: An in vivo preliminary study in a porcine model.

PURPOSE: Several studies have evaluated the interest of Low Intensity Pulsed Ultrasound (LIPUS) in the osseointegration of dental implants in murine or rabbit models. However, the thinness and narrowness bones make it difficult to study the effect of LIPUS. The purpose of this study is to assess the ability of LIPUS to stimulate bone formation in contact with a titanium dental implant in a porcine model. METHODS: Eight adults mini-pigs were used. An implant is placed on each tibial crest in the metaphysis. The right side was treated with LIPUS at 1 MHz and 300 mW/cm2 of acoustic intensity during 15 minutes per day on 5 consecutive days and during 42 days. The left side was not treated. The Bone Volume/Total Volume ratio (BV/TV), the Intersection Surface (IS) of the volume of interest by the binarized bone and the Trabecular bone Thickness (TbTh) around the implant were analyzed. RESULTS: At 42 days, BV/TV ratio is significantly higher on the treated side (42,1+/-8,76% versus 32,31+/-10,11%, p < 0,02); as well as TbTh with 0,13+/-0,01 mm versus 0,10+/-0,01 mm (p < 0,01). IS is also significantly higher on the treated side (40,7 +/- 12,68 mm2 versus 33,68+/-9,44 mm2 at 200 µm from the implant surface; p < 0,01). CONCLUSION: The present study showed that LIPUS can significantly increase bone formation and accelerate the healing process at the bone-implant interface in a porcine model. Its low toxicity, low immunogenicity and non-invasion make it a complementary treatment of choice for improving the bone formation around titanium implants.

Lipofilling of the lower eyelids: A craniofacial tool to postpone the facial advancement in craniofacial syndromes.

PURPOSE: Many genetic diseases are responsible for a defect in the growth of the maxilla. Craniofacial syndromes such as Crouzon syndrome or Apert syndrome are typically associated with a major hypoplasia in the midface responsible for exophthalmos, leading to palpebral malocclusion and frequent corneal complications. Several treatments have been used to manage ocular protection in craniofacial syndromes such as tarsorrhaphy, a fronto-orbital advancement and/or a Lefort III osteotomy with or without distraction. We describe a new approach as a waiting solution to relieve exophthalmos: the autologous fat grafting of the lower eyelids. MATERIAL AND METHODS: Children operated from lipofilling of the lower eyelids at the Referral Center for craniosynostosis of Femme-Mère-Enfant Hospital in Lyon, were included. All the patients suffered from midface hypoplasia and exophthalmos with insufficient eyelid closure responsible of multiples episodes of keratitis and corneal ulcerations. RESULTS: Four children were included: three suffered from Crouzon Syndrome and one suffered from Apert Syndrome. The fat was reinjected at the level of the infraorbital rim, the nasolabial fold or the palpebrojugal fold, in the different planes, according to the patients' needs. Six months after surgery, a second surgery was performed for the patients whose lagophtalmos persisted. Six months after the second procedure, the results are satisfactory with a good eyelid closure. CONCLUSION: The lipofilling, well used in pediatric surgery to restore facial symmetry, can also be used, as a waiting solution or complement to treat complicated exophtalmos with visual prognosis involved in congenital craniofacial syndromes.

Osteocutaneous-flap-related osteomyelitis following mandibular reconstruction: a cohort study of an emerging and complex bone infection.

Osteocutaneous flap (OCF) mandible reconstruction is at high risk for surgical site infection. This study aimed to describe diagnosis, management, and outcome of OCF-related osteomyelitis. All patients managed at our institution for an OCF-related osteomyelitis following mandible reconstruction were included in a retrospective cohort study (2012-2019). Microbiology was described according to gold-standard surgical samples, considering all virulent pathogens, and potential contaminants if present on at least two samples. Determinants of treatment failure were assessed by logistic regression and Kaplan-Meier curve analysis. The 48 included patients (median age 60.5 (IQR, 52.4-66.6) years) benefited from OCF mandible reconstruction mostly for carcinoma ( n = 27 / 48 ; 56.3 %) or osteoradionecrosis ( n = 12 / 48 ; 25.0 %). OCF-related osteomyelitis was mostly early ( ≤ 3 months post-surgery; n = 43 / 48 ; 89.6 %), presenting with local inflammation ( n = 28 / 47 ; 59.6 %), nonunion (wound dehiscence) or sinus tract ( n = 28 / 47 ; 59.6 %), and/or bone or device exposure ( n = 21 / 47 ; 44.7 %). Main implicated pathogens were Enterobacteriaceae ( n = 25 / 41 ; 61.0 %), streptococci ( n = 22 / 41 ; 53.7 %), Staphylococcus aureus ( n = 10 / 41 ; 24.4 %), enterococci ( n = 9 / 41 ; 22.0 %), non-fermenting Gram-negative bacilli ( n = 8 / 41 ; 19.5 %), and anaerobes ( n = 8 / 41 ; 19.5 %). Thirty-nine patients (81.3 %) benefited from surgery, consisting of debridement with implant retention (DAIR) in 25 / 39 (64.1 %) cases, associated with 93 (IQR, 64-128) days of antimicrobial therapy. After a follow-up of 18 (IQR, 11-31) months, 24 / 48 (50.0 %) treatment failures were observed. Determinants of treatment outcomes were DAIR (OR, 3.333; 95 % CI, 1.020-10.898) and an early infectious disease specialist referral (OR, 0.236 if ≤ 2  weeks; 95 % CI, 0.062-0.933). OCF-related osteomyelitis following mandibular reconstruction represents difficult-to-treat infections. Our results advocate for a multidisciplinary management, including an early infectious-disease-specialist referral to manage the antimicrobial therapy driven by complex microbiological documentation.

Bilateral dermoid cysts of the upper lip.

Dermoid cysts are benign tumors found throughout the body. They are either congenital or acquired. Derived from both ectoderm and mesoderm, they are often diagnosed in childhood. Dermoid cysts of the head and neck region account for less than 10% of all dermoid cysts. The most common locations of the cysts are the periorbital region and the neck. We expose the case of a 47-year-old man who presented with bilateral dermoid cysts of the upper lip. Only 1 case of unilateral dermoid cyst of the upper lip is referenced in the literature. Authors report the first case of bilateral dermoid cysts and discuss its possible origins.

Stimulation of oral mucosal regeneration by low intensity pulsed ultrasound: an in vivo study in a porcine model.

PURPOSE: Many studies have shown the ability of low intensity pulsed ultrasound (LIPUS) to stimulate the bone, cartilage and tendon regeneration but only a few studied LIPUS interest in the regeneration of the oral mucosa. The purpose of this study is to assess the ability of LIPUS to stimulate the regeneration of the palatal mucosa in a porcine model. METHODS: Ten adults mini-pigs were used. Two mucosal wounds were realised on the left and right side of the palate of each pig. The right side was treated with LIPUS at 1 MHz of frequency and 300 mW/cm2 of acoustic intensity. The left side was not treated. The morphology of the wound was evaluated using a polymer silicone molding. RESULTS: The difference between two sides was significant from day 7 with a p value < 0.0001. At day 21, the wound is completely healed on all pigs with LIPUS. The control soft tissue defect exposed a healing of 80%. CONCLUSIONS: The present study showed that the use of LIPUS on the oral mucosa accelerates the healing of the masticatory mucosa.

Description of a novel patient with the TRPM3 recurrent p.Val837Met variant.

De novo heterozygous missense mutations in TRPM3 have been shown to cause developmental and epileptic encephalopathies (DEE). It is a very rare condition, as only 9 patients have been described to date. We report here a novel patient carrying the recurrent p.Val837Met variant and presenting new clinical features, such as trigonocephaly, expanding the phenotypical spectrum of the disease.

Metopic triangle osteotomy in trigonocephaly Piezosurgery: a technical variant to control metopic emissary veins.

Premature fusion of the metopic suture results in trigonocephaly with variable degrees of anterior cranial fossa dysmorphia and craniofacial deformity. Different surgical corrective techniques that aim to reshape the forehead and enlarge the cranial volume have been described. Typical variations of the standard fronto-orbitary advancement carry the risk of relevant blood loss during frontal osteotomy, where paired emissary metopic veins are disrupted. The authors present a technical variant that preserves a bony triangle over the glabella to optimize control of these veins, which represent the major source of bleeding, and applies Piezosurgery to perform the osteotomies to minimize bone substance loss. The video can be found here: https://vimeo.com/511536423.