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.

Mandibular Condyle Osteoma.

BACKGROUND: Osteoma is a benign tumor composed of both cortical and cancellous bones that increase in size with continuous formation of bone. The pathogenesis is unknown. Osteomas can cause symptoms depending on their location and size. They can be asymptomatic or symptomatic, with trismus, limitation of mouth opening, and progressive malocclusion with facial asymmetry and can be painful. AIM: The aim of this paper is to report an unusual case of osteoma in the mandibular condylar neck and review the cases of mandibular condyle osteomas that have been reported in the last 15 years. CONCLUSIONS: Only a few cases involving the temporomandibular joint have been reported. We report an unusual case of osteoma in the mandibular condylar neck causing restricted mouth opening in addition to pain. Complete surgical excision in symptomatic cases is the therapy of choice with a low recurrence rate.

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.

The Buccal Fat Pad for Closure of Oroantral Communication.

The buccal fat pad (BFP) is a well-established tool in oral and maxillofacial surgery and its use has proved of value for the closure of oroantral communications. Oroantral communication may be a common complication after sequestrectomy in "bisphosphonate-related osteonecrosis of the jaws."The authors report a clinical case of a 70-year-old female patient in bisphosphonate therapy presented with right maxillary sinusitis and oroantral communication after implants insertion.The BFP was used to close the defect. The patient had an uneventful postoperative healing without dehiscence, infection, and necrosis.The authors postulate that the primary closure of the site with BFP may ensure a sufficient blood supply and adequate protection for an effective bone-healing response to occur.

Selective augmentation of stem cell populations in structural fat grafts for maxillofacial surgery.

Structural fat grafting utilizes the centrifugation of liposuction aspirates to create a graded density of adipose tissue. This study was performed to qualitatively investigate the effects of centrifugation on stem cells present in adipose tissue. Liposuction aspirates were obtained from healthy donors and either not centrifuged or centrifuged at 1,800 rpm for 3 minutes. The obtained fat volumes were divided into three layers and then analyzed. The results demonstrate that centrifugation induces a different distribution of stem cells in the three layers. The high-density layer displays the highest expression of mesenchymal stem cell and endothelial markers. The low-density layer exhibits an enrichment of multipotent stem cells. We conclude that appropriate centrifugation concentrates stem cells. This finding may influence the clinical practice of liposuction aspirate centrifugation and enhance graft uptake.

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.

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.

New mini-osteotomy of the infraorbital nerve in bony decompression for endocrine orbitopathy.

Endocrine orbitopathy is a systemic complex disease that involves the orbital contents. The symptoms are exophthalmos and correlated. The surgical techniques used to correct this condition can be fat decompression by the Olivari technique, 3-wall bony decompression, or the combination of these 2 surgical strategies, the ancillary procedure. Fat decompression is indicated when the intraconal and extraconal fat tissue is increased, whereas bony decompression is used in the presence of extraocular muscle involvement, associated with a normal quantity of intraconal-extraconal fat. Surgical techniques include the transconjunctival approach and ostectomy of the medial wall (when possible through endoscopy), orbital floor, and lateral wall of the orbit.Complications of this type of intervention are often represented by sensitivity disorders of the second branch of the trigeminal nerve, compressed by the intraorbital contents when they prolapse into the sinus. Possible sensitivity disorders are paresthesia, anesthesia, hypoaesthesia, dysesthesia, and hyperesthesia.The innovation introduced by the first author in 2007 consists of a mini ostectomy around the infraorbital foramen with removal of bone fragment. This determines relaxation of the nerve and makes easier the descent toward the sinus, allowing a larger expansion of the orbit contents. The absence of compression significantly reduces the sensitive complications. After treatment of the basic disease, surgical indications should be given according to the Werner classification. Fat decompression with the coronal approach is almost entirely abandoned for the transconjunctival approach, which allows adequate exposure of the lower orbit.The use of mini ostectomy of the infraorbital foramen combined with a 3-wall bony expansion showed a significant reduction of sensitive complications that often cause patient discomfort.

Early surgical treatment in unilateral coronoid hyperplasia and facial asymmetry.

Unilateral coronoid hyperplasia is a rare condition in the pediatric age. It may be an unrecognized cause of restricted mouth opening in children.The limited jaw movement is due to the enlargement of the coronoid process of the mandible that impinges on the zygomatic arch during mouth opening. This pathologic condition is still unknown and often misdiagnosed.Although in the past the term osteochondroma has been used to describe most of the unilateral and a few of the bilateral cases, there is no histologic evidence that the process has a neoplastic origin.Microscopic examination of the removed coronoid process has revealed hyperplastic compact bone covered with a thin layer of normal cartilage.There are multiple causes of mandibular hypomobility, each of them associated with different anatomic structures and etiologies, and a large number of cases, mostly bilateral, are idiopathic in nature.Several theories of pathogenesis have been proposed: temporomandibular joint dysfunctions, mandibular hypomobility, temporalis hyperactivity, hormonal stimulus, persistent cartilage growth center, genetic inheritance, and family factors.Unilateral coronoid hyperplasia is usually due to a trauma or a pathologic condition and is associated with facial asymmetry, being more frequently seen in women with histologic chondromatous or neoplastic changes. A thorough clinical history should include information about the onset and progression of pain and other subjective symptoms.In this study, we present a case of unilateral hyperplasia of the coronoid process in a 3 year-old female who, to the best of our knowledge, is the youngest patient so far reported with such anomaly.Our findings support the recommendation that early surgical treatment and aggressive postoperative physical therapy should be taken into account to allow for recovery of morphology and growth function in children.

Anterior skull base fractures: guidelines for treatment.

Cranial base fractures still represent a challenging issue. A multidisciplinary approach and the contribution of different specialists is mandatory. There is still a controversy regarding the correct approach to these trauma due to the diversity of opinions as well as surgical approaches and timing.

Posttraumatic enophthalmos: etiology, principles of reconstruction, and correction.

Enophthalmos is defined as a backward, and usually downward, displacement of the globe into the bony orbit. In posttraumatic enophthalmos, the mechanisms that determine globe position are: 1) the enlargement of the orbital cavity; 2) the herniation of orbital fat into the maxillary sinus; and 3) fat atrophy, loss of ligament support, and scar contracture. The aim of this article is to analyze the strategies to prevent enophthalmos and to correct late posttraumatic enophthalmos. In this study, 80 patients (52 cases of orbitozygomatic fractures and 28 late posttraumatic enophthalmos) were treated between January 1998 and January 2005. Fracture reduction in primary enophthalmos was performed. In enophthalmos as sequelae, the treatment consisted of orbital reconstruction in combination with bone grafts harvested from calvaria, iliac crest, and/or orbital osteotomies. In some cases, biomaterials were also used. All these techniques may also be combined depending on the severity of enophthalmos. The results were satisfactory in all cases. It is evident that a perfect correction of the deformity is difficult to achieve. Often soft tissue changes limit the aesthetic and morphologic results, despite adequate bony reconstruction.