The arrhinias: Proboscis lateralis literature review and surgical update.

Proboscis lateralis (PL) is a rare malformation, reported for the first time in 1861 by Forster in his monograph on congenital malformations of the human body. The abnormal side of the nose is represented by a tube-like rudimentary nasal structure, attached at any point along the embryonic fusion line between the anterior maxilla and the frontonasal processes. As clefts of the lip (and alveolus) are bilateral or unilateral, an arrhinia can be bilateral (total) or unilateral. In this case it is a 'hemi-arrhinia' (or heminasal agenesis. The arrhinias represent three groups of anomalies, each with different levels of clinical severity, some involving association with the labio-palatal cleft or agenesia of the premaxilla (1). In PL the nasal cavity on the affected side is replaced by a tubular appendage located off-center from the midline of the face, arising commonly from the medial aspect of the roof of the orbit (2). It is usually associated with heminasal aplasia or hypoplasia, microphthalmia, and - less commonly - with midline clefting. Associated brain and cranial vault anomalies are seen in 19% of these patients. PL is usually unilateral, with very few symmetrical/bilateral cases being reported (3). Morpho-aesthetic and psychological problems are frequent concerns for the patients and their families. In this study, the authors describe a clinical case and the chosen surgical technique, as well as reviewing the alternative techniques present in the literature.

Craniomaxillofacial Fibrous Dysplasia: Conservative Treatment and Maxillary Osteotomy Using the Schuchardt-Kufner Technique.

Fibrous dysplasia (FD) is a disturbance of the mesenchymal tissue that accounts for 2.5% of all bone tumors and more than 7% of nonmalignant bone tumors. In the craniomaxillofacial region, FD affects the calvaria, skull base, zygoma, and jaws, the prevalent site being the maxilla (50% of cases). Therapy for craniomaxillofacial FD is surgical. The goals of surgery are to prevent functional disorders and restore facial symmetry, volume, and contour. In this article, we present a case of a young female patient affected by right orbital-zygomatic-maxillary FD. She had developed facial asymmetry and malocclusion that were corrected using the Schuchardt-Kufner osteotomy technique.

Temporomandibular Joint Ankylosis After Early Mandibular Distraction Osteogenesis: A New Syndrome?

Distraction osteogenesis (DO) has been one of the most innovative concepts in cranio-maxillofacial syndromology and surgery over the last 25 years.Early mandibular distraction in severe micrognathia has recently been recognized as an effective treatment option to safely relieve upper airway obstruction associated with mandibular deficiency.An increased incidence in temporomandibular joint complications during DO in neonates has recently been reported, especially in syndromic patients.The authors report 2 children affected by severe micrognathia and severe respiratory distress at birth.Early DO was performed during the first 2 months of the life in another institution with the aim of increasing mandibular length and upper airway size.Both the patients had severe restricted jaw opening after DO and mandibular abnormalities.Temporomandibular joint ankylosis after early mandibular distraction could be a considered a new pathological entity.

Combined Endoscopic and Trans Palpebral Orbital Reconstruction for Silent Sinus Syndrome.

Silent Sinus Syndrome is defined as a painless spontaneous and progressive enophthalmos and hypoglobus with maxillary sinus hypoplasia and orbital floor resorption. It is caused by maxillary sinus atelectasis in a setting of ipsilateral chronic maxillary sinus hypoventilation. The syndrome was first described in 1964 by Montgomery, but the term "Silent Sinus Syndrome" was not coined until 1994 by Soparkar. The aetiology is still controversial: some authors postulate a basal hypoplastic sinus, other suggest an acquired process due to an obstruction of the ostium in the medium meatus. Silent Sinus Syndrome presents in the third to fifth decades of life, very rarely in childhood with no gender predilection and it is usually a unilateral disorder. The symptoms are not shown to be related to chronic sinuses disease. The clinical signs are: enophthalmos, hypoglobus, upper lid retraction secondary to dystopia of the globe, sinking of the eye and orbital asymmetry, deepened upper lid sulcus, disappearance of the palpebral fold line, lagophthalmos, vertical diplopia, malar depression, and facial asymmetry. Extraocular muscle function is generally preserved and usually there is no visual impairment. The diagnosis is confirmed by computed tomography scan of the orbits and paranasal sinuses. The treatment consists of orbital reconstruction and functional rehabilitation of the maxillary sinuses.

Multiple mandibular fractures. Treatment outlines.

Multiple mandibular comminuted fractures usually occur in high energy traumas. The authors describe the management and treatment of multiple mandibular fractures in a young patient after a suicide attempt.

Piezosurgery: A new and safe technique for distraction osteogenesis in Pierre Robin sequence review of the literature and case report.

INTRODUCTION: Pierre Robin sequence (PRS) is characterized by microgenia and retrognathia. Cleft palate and glossoptosis are frequently associated with airway obstruction and difficulty in swallowing. Distraction osteogenesis with micro-distractors has recently been considered as a surgical option during the neonatal age. CASE PRESENTATION: A 6-week-old female with PRS underwent mandibular lengthening in neonatal age. Mandibular osteotomies were performed with the piezoelectric scalpel. DISCUSSION: Piezosurgery represents an innovative technique as it offers the maxillofacial surgeon the opportunity to make precise bone cuts without damaging the soft tissue, minimizing the invasiveness of the surgical procedure, and the opportunity of working in a field which is almost totally blood free. CONCLUSION: The use of a piezoelectric device to perform this kind of surgery provides clinical and surgical results which would be difficult with traditional instruments, not only for the patient's benefit but also for the surgeon's. Preservation of the original bony structure, especially of the cancellous bone, will benefit the bone healing process due to its high estrogenic potential.

Three-dimensional volumetric restoration by structural fat grafting.

The use of adipose tissue transfer for correction of maxillofacial defects was reported for the first time at the end of the 19th century. Structural fat grafting (SFG) was introduced as a way to improve facial esthetics and in recent years has evolved into applications in craniomaxillofacial reconstructive surgery. Several techniques have been proposed for harvesting and grafting the fat. However, owing to the damage of many adipocytes during these maneuvers, the results have not been satisfactory and have required several fat injection procedures for small corrections. The author's (L.C.) overview the application of SFG in the management of volumetric deficit in the craniomaxillofacial in patients treated with a long-term follow-up.

Management of temporomandibular joint reankylosis in syndromic patients corrected with joint prostheses: surgical and rehabilitation protocols.

Temporomandibular joint ankylosis (TMJA) is a severe disorder described as an intracapsular union of the disc-condyle complex to the temporal articular surface with bony fusion. The management of this disability is challenging and rarely based on surgical and rehabilitation protocols. We describe the treatment in two young adults affected by Goldenhar syndrome and Pierre Robin sequence with reankylosis after previous surgical treatments. There are three main surgical procedures for the treatment of TMJA: gap arthroplasty, interpositional arthroplasty, and joint reconstruction. Various authors have described reankylosis as a frequent event after treatment. Treatment failure could be associated with surgical errors and/or inadequate intensive postoperative physiotherapy. Surgical treatment should be individually tailored and adequate postoperative physiotherapy protocol is mandatory for success.

Cranial bone grafts in forehead reconstruction after resection for benign tumors.

Cranial bone graft was first used in forehead reconstruction by Muller and König as early as 1890. Because cranial bone graft is the ideal material for almost all facial and skull repairs, surgeons have subsequently used this technique to repair skull defects. In fact, membranous bone (calvaria) is superior to endochondral bone (ilium, rib) and maintains its volume to a significantly greater extent than endochondral bone.The authors, after reviewing the literature, report 3 cases of forehead benign tumors treated by resection and primary reconstruction using cranial bone grafts. The preoperative computed tomographic scanning should lead to appropriate diagnosis and treatment planning, which includes total excision and primary bone grafting of the defect to prevent soft-tissue contraction.

Craniofacial surgical strategies for the correction of pneumosinus dilatans frontalis.

INTRODUCTION: Pneumosinus dilatans is a rare condition and different techniques have been proposed for its management and correction. The abnormally expanded, aerated frontal sinus has been described in the literature as: frontal sinus hypertrophy, pneumosinus dilatans, pneumosinus frontalis, aerocele, pneumocele, sinus ectasia, hyperpneumatization and others. The precise aetiology and pathogenesis of the condition is unknown, although several basic hypotheses have been proposed MATERIAL AND METHODS: The authors report two cases of frontal bossing and supraorbital ridge deformity correction using craniofacial surgical principles. DISCUSSION: Functional and morphological results are discussed and compared with other open procedures. CONCLUSION: A variety of surgical procedures have been proposed for the correction of the pneumosinus dilatans frontalis. The craniofacial approach is advocated to reproduce the normal anatomy of the forehead in the upper part, the supraorbital rim and glabellar area.

Contouring of the forehead irregularities (washboard effect) with bone biomaterial.

Calvarial vault defects may be repaired with autologous bone or alloplastic materials, such as methyl methacrylate, hydroxyapatite, titanium, or porous polyethylene. The criterion standard for repairing small cranial defects is autogenous bone from iliac crest or split calvarial grafts. However, autogenous grafts may result in donor-site morbidity, increased operative time, reabsorption, blood loss, and additional time for recovery. An alloplastic material should have some ideal properties, including easy adaptation, biocompatibility, which permit ingrowth of new tissue, stability of shape, and low rate of reabsorption. An implant in this area should be easily shaped and positioned, allowing an easy tissue in growth.The authors report the case of a 50-year-old man with a deformity of the frontal region as a result of a frontonaso-orbitoethmoidal fracture after reduction and fixation of the fractures and right frontal sinus cranialization with frontal craniotomy via coronal approach. The deformity caused the typical aspect (washboard effect). Correction and reconstruction were performed by using Cerament (Bonesupport AB, Lund, Sweden), alloplastic biphasic material, composed of 60% α-hemihydrate of calcium sulfate and 40% hydroxyapatite. Four years after the surgery, the patient had recovered with satisfactory morphology of the forehead as well as disappearance of the frowning look in the frontal region.

Frontal linear scleroderma: long-term result in volumetric restoration of the fronto-orbital area by structural fat grafting.

Frontal linear scleroderma (also known as "en coup de sabre") is a congenital deformity characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal area. The authors present a case of a 34-year-old woman with history of en coup de sabre. In different steps, volumetric restoration of the fronto-orbital region has been obtained by structural fat grafting technique. After 3 reconstructive surgeries, morphologic, functional, and aesthetic long-term results have been obtained.

Surgical decompression in endocrine orbitopathy. Visual evoked potential evaluation and effect on the optic nerve.

Endocrine orbitopathy (EO) represents the most frequent and important extrathyroidal stigma of Graves disease. This chronic autoimmune condition involves the orbital contents, including extraocular muscles, periorbital connective-fatty tissue and lacrimal gland. The increase of fat tissue and the enlargement of extraocular muscles within the bony confines of the orbit leads to proptosis, and in the most severe cases optic neuropathy, caused by compression and stretching of the optic nerve. The congestion and the pressure of the enlarged muscles, constrict the nerve and can lead to reduced sight or loss of vision with the so called "orbital apex syndrome". Generally surgical treatment of EO, based on fat and/or orbital wall expansion, is possible and effective in improving exophthalmos and diplopia. Since there are limited reports focussing on optic neuropathy recovery after fat and/or orbital walls decompression the Authors decided to perform a retrospective analysis on a series of patients affected by EO. The study population was composed of 10 patients affected by EO and presenting to the Unit of Cranio Maxillofacial Surgery, Center for Craniofacial Deformities & Orbital Surgery St. Anna Hospital and University, Ferrara, Italy, for evaluation and treatment. A complete Visual Evoked Potentials (VEP) evaluation was performed. There were seven women and three men with a median age of 55 years. Optic nerve VEP amplitude and latency were recorded as normal or pathological. Abnormal results were scored as moderate, mild and severe. Differences in VEP pre and post-operatively were recorded as present or absent (i.e. VEP Delta). Pearson chi square test was applied. There were 20 operated orbits. The first VEP evaluation was performed 3.2 months before surgery and post-operative VEP control was done after a mean of 18.7 months. Fat decompression was performed in all cases and eight patients had also bony decompression. VEP amplitude and latency were affected in 10 and 15 cases before operation and six and nine after surgery, respectively. VEP amplitude and latency significantly improved after orbital decompression. Fat and orbital wall decompression are of paramount importance not only to improve exophthalmos and diplopia in patients affected by EO but also as rescue surgery for severe cases where optic neuropathy caused by stretching of the optical nerve is detected by VEP. Imaging and functional nerve evaluation are mandatory in all cases of EO.

Endocrine orbitopathy: 11 years retrospective study and review of 102 patients & 196 orbits.

BACKGROUND: Endocrine Orbitopathy (EO) is the most frequent and important extrathyroidal stigma of Graves' disease. In the active stage of the orbitopathy fibrosis and hypertrophy of the extra-ocular muscles can lead to visual impairment and diplopia. In the stable phase of the disease surgical treatment by orbital expansion and/or orbital decompression can improve the quality of life and it is indicated for morpho-aesthetic and functional reasons. METHODS: From 1998 to 2009 a consecutive series of 131 patients (251 orbits) with endocrine orbitopathy underwent surgery by different techniques. The medical records of 102 patients (78%) and 196 orbits were available to be assessed retrospectively. Ninety-four patients had bilateral involvement of the orbits and eight unilateral. A total of 556 operations were performed. RESULTS: Mean pre-operative exophthalmos was 24.7 ± 2.5 mm (max-min 20-34), mean post-operative exophthalmos was 21 ± 1.8 mm (max-min 18-26), and mean differential exophthalmos was 3.9 ± 1.7 mm (max-min 1-9). The reduction in exophthalmos after surgery had a mean value of 3.8 mm (min 1, max 9). Kaplan Meier algorithm demonstrates that intra-operative cortisone injection had an adverse effect on post-operative diplopia. CONCLUSIONS: The surgical technique used should be adapted to the individual patients' needs. In severe cases intraorbital fat removal and bony decompression can be and carried out in one surgical procedure. An integrated global approach by a multidisciplinary team is strongly recommended. Strabismus surgery is a significant part of the overall treatment. The Authors suggest general surgical guidelines and an algorithm of treatment in EO.

Structural fat grafting: facial volumetric restoration in complex reconstructive surgery.

BACKGROUND: The authors overview the application of structural fat grafting (SFG) in the management of volumetric deficit in the maxillofacial area. Structural fat grafting was introduced as a way to improve facial aesthetics and in recent years has evolved into applications in craniomaxillofacial reconstructive surgery. METHODS: A retrospective cohort study population was composed of patients grafted with autologous fat referred to our department from February 2005 to July 2009. Each patient was operated on with SFG technique according to Coleman. Subjects were screened to these possessing an atrophy of facial soft tissues after trauma, tumor resection, congenital deformities and clefts, Parry-Romberg and scleroderma, orbital and periorbital surgery, facial palsy, burns, and scars. RESULTS: Forty-seven patients (27 females and 20 males) with a mean age of 38 years (minimum, 15 years; maximum, 71 years) were enrolled in the current study. The mean postoperative follow-up was 14 months. A total of 548 sites were grafted into 47 patients: malar, n = 103; nasolabial fold, n = 82; lip, n = 86; eyebrow, n = 32; jaw line, n = 18; philtrum, n = 19; forehead, n = 33; temple, n = 34; eyelid, n = 70; chin, n = 16; cheek, n = 25; nose, n = 23; and neck scar, n = 7. Each patient was operated on 1.6 times, and 11.6 was the average number of grafted sites. CONCLUSIONS: The authors have performed 548 procedures of SFG in 47 patients with good results as well as improvement in facial morphology, function, shape, and volume and improvement in the patients' appearance.

Patholologic fracture of the mandible after removal of follicular cyst.

OBJECTIVE: The objective of the study was to report a 61-year-old man who presented a complication after mandibular follicular cyst removal. METHODS: The patient underwent surgery, via intraoral approach: removal of the lesion and the dental follicle with curettage and extraction of the mandibular right third molar. Two weeks after surgery, the patient reported a slight malocclusion. The x-ray showed a fracture near the right mandibular angle. The fracture was treated by application of a long mandibular plate. RESULTS: Two years after surgery, the patient is asymptomatic. Inferior alveolar nerve sensitivity returned completely. CONCLUSIONS: In the reported case, a satisfactory result was obtained after the treatment of the complication confirmed by postoperative x-rays.

Endocrine orbitopathy (Graves disease): transpalpebral fat decompression in combination with 3-wall bony expansion.

Endocrine orbitopathy (EO) is a chronic, multisystem autoimmune disorder caused by lymphocyte infiltration, edema, and proliferation of endo-orbital connective tissue. These conditions involve the extraocular muscles, intraconal and extraconal fat, and, to a lesser extent, the lacrimal gland.Endocrine orbitopathy may be associated with toxic diffuse goiter and/or pretibial myxedema (Graves disease) and may appear without alterations in thyroid function (euthyroidism). It is characterized by antibodies that stimulate a general fibroblastic reaction (thyroid gland and lower extremities) and involves orbital fat tissue and muscles. The clinical signs and symptoms of EO reflect the mechanical consequences of increased orbital tissue volume and pressure within the orbit. Endocrine orbitopathy is marked by chronic evolution and, at times, a malignant outcome.

Parry-Romberg syndrome: volumetric regeneration by structural fat grafting technique.

The use of adipose tissue transfer for correction of maxillo-facial defects was reported for the first time at the end of the 19th century and has since been the subject of numerous studies. Structural Fat Grafting (SFG) differs from other fat grafting techniques in both the harvesting and placement of the fat. The main indications for SFG are for the restoration and rejuvenation of the face. Recent applications include the correction of localised tissue atrophy, loss of substance due to trauma, post-tumour, congenital complex craniofacial deformities, burns, and hemifacial atrophy. The authors describe a case of a 20-year-old woman with right Parry-Romberg syndrome (PRS) treated over many years with many different surgical reconstructive techniques with poor results. After five SFG (three complete procedures and two minor revisions) over three years, the authors obtained a good aesthetic result with complete patient satisfaction. SFG can be an excellent technique for facial reconstruction and re-contouring, with natural and long-lasting results.