The role of the dentist and the orthodontist in early diagnosis of Gorlin-Goltz syndrome: a cephalometric and photometric study.

AIM: The aim of this study was to analyse the facial characteristics and the craniofacial morphology in GGS patients in order to enable an early diagnosis. BACKGROUND: Gorlin-Goltz syndrome (GGS) is a autosomic dominant disease, characterised by basal cell carcinoma, palmar/plantar pits, maxillary and mandibular keratocysts and dental abnormalities. METHODS: Nine out of a sample of 24 GGS patients had complete cephalometric and photographic records at an average age of 8.7 years. Cephalometric and photometric analysis were carried out with standard analyses and compared with healthy patients matched for sex and age. CONCLUSION: Early diagnosis of GGS based on clinical features could be useful to identify the presence of keratocysts through x-ray examination proceeding with surgical removal at an early stage, limiting space occupying damages.

Nasoalveolar molding and skeletal development in patients with bilateral cleft lip and palate: A retrospective cephalometric study at the completion of growth.

The aim of this retrospective long-term study was to assess the influence of primary columella lengthening and presurgical nasoalveolar molding (NAM) on the skeletal development at the completion of growth in patients with bilateral cleft lip and palate (BCLP). Lateral cephalometric radiographs at the completion of growth of consecutively treated patients BCLP patients, operated by the same surgeon, who had undergone NAM were compared with a second group of BCLP patients who were not treated with NAM. The groups were matched for sex and age. Independent samples t tests were carried out. 23 Lateral cephalometric radiographs of BCLP patients (mean age 18.2 ± 1.3 years) who had undergone NAM were compared with a second group of 23 BCLP patients (mean age 18.4 ± 1.3 years) who were not treated with NAM. The only two significant differences were observed in Ans-Me/N-Me (control group = 0.6 ± 0.02; sample group = 0.57 ± 0.05; p = 0.019) and ILs^AnsPns (control group = 105.5 ± 7.9; sample group = 112.4 ± 8.6; p = 0.007). No other significant differences were observed in terms of facial skeletal development between the two groups. Presurgical NAM performed during infancy in BCLP patients does not seem to have negative effects on the skeletal development at the completion of craniofacial growth compared to the group of patients treated without NAM.

Non-surgical treatment of vertical excess of the premaxilla in growing bilateral cleft lip and palate patients.

Patients with bilateral complete cleft lip and palate (BCLP) may present a vertical excess of the premaxilla in childhood. This is a severe functional and aesthetic problem, where bone grafting is more challenging. The aim of this study was to describe a simple and reproducible non-surgical orthopaedic treatment for vertical excess of the premaxilla in the deciduous/early mixed dentition phase in BCLP patients. Six growing patients with complete BCLP with a severe vertical excess of the premaxilla were included. An intrusion device associated with a bonded rapid palatal expander was applied to intrude the premaxilla. Radiographic and photographic records obtained before and at the end of the orthodontic intrusion, at short- and long-term follow-up, were available. A flattening of the occlusal plane was achieved in all patients. Normalization of the position of the maxillary incisors and gingival display in relation to the upper lip was obtained, and an improvement in anterior nasal spine position was also observed in all cases. The novel technique described might be of assistance in treating BCLP children with vertical excess of the premaxilla during the deciduous/early mixed dentition phase. This simplified, easily reproducible method may allow the burden of care of this rare but complex problem affecting BCLP patients to be reduced significantly.

Maxillary shape at the end of puberty in operated unilateral cleft lip and palate: A geometric morphometric assessment using computer tomography.

INTRODUCTION: Cleft lip and palate (CLP) surgery interferes with maxillary growth and contributes to transversal and sagittal growth impairment. Our retrospective descriptive study aimed to evaluate maxillary bone shape in a homogenous unilateral CLP patient group using geometric morphometrics based on CT-scan data. MATERIAL AND METHODS: We included all patients with available CT-scans at the end of pubertal growth and operated on at Smile House of Milan, according to the standard protocol, involving two surgical steps: (1) primary closure of the lip and soft palate at 6 months of age and (2) early secondary gingivoalveoloplasty (GAP) associated with hard palate repair at 18-36 months. Shape differences between CLP and an age-matched control group were characterized using geometric morphometrics based on 15 3D landmarks. RESULTS: We included 16 unilateral CLP patients and 20 age-matched controls. Principal component and canonical variate analyses showed that the maxillary shape in CLP was significantly different from controls but that this difference was limited. Linear and angular measurements confirmed these differences. CONCLUSION: Early secondary GAP results in satisfactory maxillary shape, with significant but limited differences relative to controls.

Muscle fiber diameter assessment in cleft lip using image processing.

OBJECTIVE: To pilot investigation of muscle fiber diameter (MFD) on medial and lateral sides of the cleft in 18 infants with cleft lip with or without cleft palate (CL/P) using image processing. MATERIAL AND METHODS: Formalin-fixed paraffin-embedded (FFPE) tissue samples from the medial and lateral sides of the cleft were analyzed for MFD using an image-processing program (ImageJ). For within-case comparison, a paired Student's t test was performed. For comparisons between classes, an unpaired t test was used. RESULTS: Image processing enabled rapid measurement of MFD with majority of fibers showing diameter between 6 and 11 µm. There was no significant difference in mean MFD between the medial and lateral sides, or between CL and CLP. However, we found a significant difference on the medial side (p = .032) between males and females. CONCLUSION: The image processing on FFPE tissues resulted in easy quantification of MFD with finding of a smaller MFD on the medial side in males suggesting possible differences in orbicularis oris (OO) muscle between the two sexes in CL that warrants replication using larger number of cases. Moreover, this finding can aid subclinical phenotyping and potentially in the restoration of the anatomy and function of the upper lip.

Prevention of bisphosphonate-related mandibular fractures.

Mandibular fracture is usually the clinical end of bisphosphonate-related osteonecrosis of the jaw. This is a painful complication and patients cannot feed as usual, with a worsening of their quality of life. The goal of treatment in bisphosphonate related osteonecrosis of jaw (BRONJ) patients is to slow progression of bone necrosis. We present a novel technique for treatment of severe mandibular BRONJ in stage 3 patients that present with a high risk to develop fracture, since they have a residual unaffected mandibular bone height less than 6 mm. We treated 10 patients in this clinical situation with an extra-oral application of a reconstructive plate superficial to the platysma, to keep the plate separated from the infected site to avoid contamination and consequent need of removal, followed by an intraoral approach for active curettage of mandibular necrosis. The preservation of blood supply to the mandible and avoidance of direct contact of the infected site with the reconstructive plate are some advantages of this technique. This plate allows enhancement of mandibular strength, allowing proper treatment of the BRONJ site on the oral side without fear of causing a mandibular fracture when the residual mandible is thin. This technical solution guarantees these patients an extended disease-free period since it is effective in preventing mandibular fractures in patients with low mandibular residual height left after the BRONJ onset.

Technical refinements in mandibular reconstruction with free fibula flaps: outcome-oriented retrospective review of 99 cases.

Congenital disease, major trauma, tumour resection and biphosphonate-related osteonecrosis can lead to partial, subtotal, or total loss of the mandibular bone. Minor defects can be easily reconstructed using bone grafts, whereas microvascular free tissue transfer may be unavoidable in the case of major bone loss or poor quality of soft tissue. Simple bone or composite osteocutaneous fibula free flaps have proven invaluable and remain the workhorse for microvascular mandibular reconstruction in daily practice. Our experience with 99 consecutive fibular free flaps confirms the available data in terms of high success rate. In these cases, 90% had total success, while 7 had complete flap failures. Three of our patients showed skin paddle necrosis with bony conservation. This report focuses on the technical refinements used by the authors that can prove valuable in obtaining predictable and precise results: in particular, we discuss surgical techniques that avoid vascular pedicle ossification by removing the fibular periosteum from the vascular pedicle itself and reduce donor site morbidity and aid in management of the position in the new condylar fossa. Finally, new technologies such as intraoperative CT and custom premodelled fixation plates may also increase the predictability of morpho-functional results.

The park-bench position in cervico-facial reconstructive surgery: a technical note.

Resection and simultaneous reconstruction with free flaps of wide tumours of the scalp and posterior neck region are difficult to perform through a single surgical approach. In such cases, the park-bench position - a lateral oblique position - could allow simultaneous resection and reconstruction of tumours of the scalp and occipital region without changing the patient's position. In the case described here, this position was used to treat microcystic adnexal carcinoma in a patient who presented with three scalp lesions and a lesion of the right scapular region. This arrangement allowed both resection and reconstruction with a microvascular flap without changing the patient's position, as a result of being able to find neck vessels suitable for microanastomosis. Reconstruction was carried out using a right latissimus dorsi myogenous and subcutaneous flap, and anastomoses were created between the thoracodorsal artery and vein, and the transverse cervical artery and vein. In this position, transverse cervical vessels are well placed, with good exposure, and are in an optimal location for use in microvascular surgery. At 8 months post-reconstruction, the patient was in good condition without local recurrence or distant metastases, and the flap showed a good lining and contour. In conclusion, the park-bench position facilitates surgical excision, flap harvesting and microsurgery in the same patient position.

Iliac crest fresh-frozen allografts and autografts in maxillary and mandibular reconstruction: a histologic and histomorphometric evaluation.

AIM: The aim of this paper was to compare histologically and histomorphometrically the osseointegration of iliac crest fresh-frozen allografts and autografts in human pre-prosthetic maxillary and mandibular onlay bone blocks reconstruction. METHODS: Twelve patients with edentulous atrophic ridges, scheduled for implant-supported prosthetic restorations, underwent reconstruction using iliac crest fresh-frozen allografts (group A, six patients) or autografts (group B, six patients). Four-to-nine months later implants were placed in the augmented areas and bone specimens were simultaneously obtained using trephine burs. The specimens were processed for ground sections and evaluated histologically and histomorphometrically. RESULTS: The postoperative course was uneventful in all patients in group B and in all except one in group A. Late complications occurred in 5 patients of group A. Dental implants could be inserted in all cases. Specimens from group A showed a vascularized bone with osteoprogenitor stem cells and medium-high grade of bone remodeling. Small areas of necrotic bone were observed sporadically. Sections obtained from group B revealed an advanced stage of bone remodeling. The histomorphometric analysis showed in group A a mean proportion of 24.7±14.7% for lamellar bone, 28.4±13.3% for newly formed bone and 46.9±16.9% for bone marrow; in group B the corresponding values were 25.3±15.3%, 22.9±11.0%, 51.7±15.7%. No statistically significant difference was found (Wilcoxon Test; P>0.05). CONCLUSION: There were no significant histological differences between group A and B. Larger studies with long term follow-up are needed to confirm that fresh-frozen allografts are a reliable alternative to autografts.

Autonomized flaps in secondary head and neck reconstructions.

Free flaps, with their very high rates of success and low donor site morbidity, are considered the gold standard in head and neck reconstruction, allowing the transfer of ideal tissues for head and neck reconstruction. Nonetheless, under certain circumstances they may be contraindicated or cannot be utilized. We describe four subjects in which delayed locoregional flaps were used to reconstruct head and neck defects after a previous flap failure. Due to adverse anatomic and systemic conditions these patients were not suitable for a free flap, and thus one delayed prelaminated temporalis fasciocutaneous flap placement and three delayed supraclavicular flap (one of which was prelaminated) placements to reconstruct large defects of the cheek and commissural region needed to be performed. All flaps and grafts were viable. All patients in this case series had acceptable functional and aesthetic outcomes. Donor-site morbidity was negligible. Delayed locoregional flap placement required a total of three surgical sessions. Although limited, our experience suggests that in cases in which a free flap is contraindicated or not ideal, locoregional flaps may be a valid and safe alternative. Limitations of these procedures include increased duration of hospitalization and, foremost, the need for three-step surgery.

Lingual nerve lesion during ranula surgical treatment: case report.

Iatrogenic lingual nerve (LN) injuries are quite common in oral surgery both in maxillo-facial surgery and in oral surgery. LN runs superficially into the lateral mouth floor just beneath the mucous layer and this position enhances damage frequency. This article lists the different aetiologies of iatrogenic LN injuries and it almost focuses on lesions due to surgical treatment of ranulas. In the case report a LN lesion due to oral ranula excision is discussed; the patient experienced anaesthesia and hyperpatia in the corrisponded tongue side. It was treated with a microneurosugical anastomosis of LN, after amputation neuroma excision. The partial and definitive recovery of perception happened in six months and was deemed satisfying with 70% of functionality restored (results compared with the functionality of the contralateral side). An algorithm for diagnosis and therapy indication for iatrogenic injuries to nerves is also proposed. In case of surgical treatment, funcitonal recovery manifests after 4-6 month; a functional recovery of 70% of total nerve function is possible. The variable that most affects nerve functional recovery is surgical treatment timing; it must be performed as soon as possible.

Mesenchymal phosphaturic neoplasm in the maxillary sinus: a case report.

The authors describe a case of oncogenic osteomalacia due to a mesenchymal phosphaturic tumour in the maxillary sinus. This is a paraneoplastic syndrome in which a tumour produces a peptide hormone-like substance (phosphatonin) that causes a urinary loss of phosphates resulting in a debilitating systemic condition. In this case, the patient experienced muscle stiffness, reduction of muscle tone, loss of weight and pathological fractures. Clinical and radiological examination revealed a tumour in the right maxillary sinus; all other results were negative. The diagnosis following pathology examination was mesenchymal phosphaturic tumour with a haemangiopericytoma-like vascular pattern. Different histological types of mesenchymal tumours can be associated with paraneoplastic syndrome, but their localization in the paranasal sinuses is rare. The correct diagnosis allows the appropriate therapeutic approach, which can lead to an almost immediate resolution of the clinical situation after surgical removal of the neoplasm as in the present case. Oncogenic osteomalacia is rare, particularly in the maxillofacial region, and only a few cases have been reported.

Pilomatrix carcinoma with visceral metastases: case report and review of the literature.

Pilomatrix carcinoma, the malignant equivalent of pilomatrixoma, is rare among skin cancers. In the literature, there have been 80 cases of pilomatrix carcinoma reported, and among them nine were with metastases. The clinical presentation of this case is suggestive for the biology and of the usual history of this neoplasm. The patient was a 53-year-old male who had been treated 2 years earlier for a pilomatrix carcinoma located in the posterior part of the neck. The clinical presentation had been characterised by sudden paraplegia caused by vertebral collapse at T4 due to bone metastases. The patient underwent a first surgery for vertebral stabilisation and medullary decompression; then, he had a second operation for the resection of the local relapse of the tumour. Literature review and analysis of this case show that the pilomatrix carcinoma should be regarded as a highly locally aggressive tumour, with a high rate of local recurrence as well as metastases.

Surgical treatment of bifid mental nerve damaged by root canal therapy. A case report.

This article describes the successful surgical treatment of a case of damage to the inferior alveolar nerve, due to inadvertent extrusion of endodontic material into the mandibular canal. The accident was favoured by an anatomical variant of the nerve canal ending with a double mental foramen, already described in the literature. The complex surgical operation of uncertain outcome was dictated by neuralgia refractory to medical treatment, rather than hypoesthesia associated with compression of the nerve trunk. Problems related to loss of sensitivity and possible causes of nerve damage (traumatic, pathological and iatrogenic) are discussed.

Pericranium graft in reconstructive surgery of atrophied maxillary bones.

In preprosthetic surgery the autologous bone is universally considered the gold standard. Calvaria is, among many options, one of the preferred for its unique characteristics of hardness, easy of harvest and very low morbidity at donor site. Moreover, it gives the possibility of harvesting the pericranium. This technique, recently introduced in common practice in Milan, allows to harvest a large quantity of periosteum to cover bone grafts perioperativly. Periosteal tissue is used to cover bone grafts for two reasons. First, it would provide a layer of tissue that, thanks to its osteogenic potential, would prevent bone resorption. Second, this would interpose a layer of soft tissue to act as a cushion between the bone and mucosal flap to minimize the risk of wound dehiscence, that would bring to bone exposure and consequent failure of reconstruction. Five jaw reconstructions were performed with autologous bone and pericranium. In all cases the outcome was good, the grafts took with correct bone volume preservation. Implants were positioned according to prosthetic needs. In one case a vascular necrosis of a mucosal flap occurred. Bone exposure was prevented by the periosteum, which was revascularized after few days, allowing bone integration. Considering its potential protective capability towards bone grafts and the lack of donor site morbidity, this technique should be considered as a standard procedure in preprosthetic reconstructive surgery.

Ossification of vascular pedicle in fibular free flaps: a report of four cases.

The fibular free flap is the most widely used flap for jaw reconstruction. Flap contouring requires removal of bone excess in the proximal segment by a subperiosteal dissection, preserving vascular connections between the pedicle and the bone and leaving well vascularized periosteum attached to the vascular pedicle. Among about 100 reconstructions with fibular flaps, 4 cases were observed of abnormal ossification along the vascular pedicle. Periosteum preserves its osteogenic capability after transposition, especially in a revascularized flap; this characteristic, together with the direct contact with the bone, allows the possibility of new bone formation along the pedicle. It would appear necessary to change the technique of reducing fibular excess, with removal of periosteum together with the bone, in order to avoid the complication described.

Transthoracic hernia after harvesting a costal and latissimus dorsi flap.

Ribs are one of the most widely used grafts in craniofacial surgery. Harvesting a costochondral graft is easy and safe. The main complications are related to pneumothorax and chest-wall deformity in children. A complication is described in a patient who underwent an orbito-zygomatic reconstruction with two contiguous ribs and a latissimus dorsi free flap, and who subsequently developed a transthoracic hernia that required reconstruction with polypropylene mesh.

The free fibula flap for treating benign mandibular lesions.

UNLABELLED: A variety of benign lesions that are typically treated conservatively can affect the mandible. The treatment must be radical when these lesions are locally aggressive and involve the perimandibular soft tissues or involve most of the thickness of the mandible. The main treatment is mandibular resection and reconstruction with bone grafts, mainly iliac crest bone grafts for segmental mandibulectomy or a calvaria bone graft for resection without interruption of the mandible body. These grafts are limited due to the possibility of infection and the unpredictable long-term resorption. Free flap surgery, particularly with fibula free flaps, represents a new era in mandibular reconstruction. This technique has similar donor site morbidity, while the transferred bone resists infection and bone resorption. These advantages are achieved at the cost of a procedure that is about 1 h longer when performed by an experienced microsurgical team. We report our experience with mandibular reconstruction following the surgical resection of benign lesions in 7 patients. All the reconstructions had good RESULTS: After reconstruction, the facial morphology showed restored symmetry of the lower third profile in all patients. The functional results were satisfactory, with restored mandibular function in all cases. No signs of recurrence have appeared in any patient after a mean follow-up of 24 months.

Primary intraosseous hemangiomas of the orbit.

INTRODUCTION: Primary intraosseus orbital hemangiomas are rare vascular anomalies, of which only 23 cases have been previously reported in the literature. We here describe a further two cases. MATERIALS AND METHODS: An anterior approach was used to remove a mass located in the inferior orbital rim in one case, and in the orbital floor in the other. RESULTS: Good restoration of the orbital walls with no signs of recurrence. DISCUSSION: Intraosseous hemangiomas of the orbit are usually located anteriorly, and can be removed by anterior surgical approaches. The main surgical problem is due to the removal of the involved areas of the orbital walls because resection without reconstruction may cause functional impairments and secondary deformities that are more damaging for the patient than the tumour itself. In order to avoid defects, the integrity of the orbital walls must be immediately restored by means of autologous bone or alloplastic grafts.