Mario Pagnoni

Hypophosphaturic mesenchymal tumor of the ethmoid associated with oncogenic osteomalacia.

Oncogenic osteomalacia is an uncommon syndrome characterized by bone pain, proximal muscle weakness, hypophosphatemia, hyperphosphaturia, and a low plasma concentration of 1,25-dihydroxy-vitamin D. The disease affects both sexes at around 40 years of age, although it can sometimes affect children and adolescents. Generally, the syndrome is associated with a tumor, usually benign, of mesenchymal origin and is resolved after removal of the tumor; this syndrome can sometimes be associated with malignant tumors. These tumors seem to be histologically heterogeneous and are generally localized in soft tissues and bone. In this article, a case of oncogenic osteomalacia associated with a hypophosphaturic mesenchymal tumor of the ethmoid is reported in a 24-year-old man. After surgical and radical removal of the tumor, the patient noted a decrease in the clinical symptoms and signs.

Orbital Volume and Surface After Le Fort III Advancement in Syndromic Craniosynostosis

Abstract There are no quantitative standards for the volumetric measurements of the orbital cavity after Le Fort III advancement. Computed tomography (CT) scan images have given the opportunity to compare with accuracy the real anatomic changes and potentially the functional improvements that resulted after a surgical treatment. Three-dimensional CT scan images processed by DICOM files in Dolphin 3D Software were used to assess orbital volume and surface in 12 subjects affected by craniofacial syndromic malformations treated with Le Fort III advancement. The preoperative (T0) and postoperative (T1: 6 months after surgery) three-dimensional craniofacial CT scans of the subjects were collected and retrospectively analyzed. Image segmentation of the anatomic orbital cavity and the three-dimensional graphic rendering were done by using the Dolphin Imaging Plus 11.0 software. The orbital volume was increased after surgery, with statistical significance, from 22,267 to 22,706.3 mm3 in the right eye and from 26,511 mm3 to 26,256.4 mm3 in the left eye. The surface of both bony orbits had an expansion, which is statistically significant. In conclusion, this study showed that the orbital advancement in white subjects after Le Fort III advancement was significant and produced a significant augmentation of the orbital volume and surface area with correction of the ocular bulb proptosis.

Le Fort III external midface distraction: surgical outcomes and skeletal stability.

Abstract A retrospective study on the stability of Le Fort III midface advancement with distraction in craniofacial dysostosis. Twenty-six surgical procedures for midface advancement were performed between 2000 and 2009. Subjects were 14 consecutive patients who underwent Le Fort III osteotomy with midface advancement using a rigid external distraction device (Synthes) in the Department of Maxillo-Facial Surgery of the University “Sapienza” of Rome. In this study, 8 patients (n = 3 females, n = 5 males) affected by craniofacial dysostosis (Crouzon, n = 5; Apert, n = 3; Pfeiffer syndrome, n = 1; and other, n = 1) were selected on the basis of age and radiologic documentation. The mean age at the time of the procedure was 8.6 years, and all the patients had at least 1 year of follow-up after distraction. The radiologic documentation of each case was composed of posteroanterior and lateral cephalograms preoperatively (T1), at the time of removal of the distractor device (T2), and at follow-up (T3). The average latency period before activation of the distractor was 5 days. The device was activated on an average of 15 mm (range, 7–22 mm) as recorded on the device at a rate of 1 mm/d. Activation period ranged from 7 to 15 days, and the consolidation period was 8 to 12 weeks. Seven anatomic landmarks and 5 cephalometric measurements were identified on the lateral cephalograms at each of the 3 time periods. The cephalograms underwent digital analysis via Dolphin imaging digitalization software by 2 operators independently. The displacement of each identified landmark was recorded and examined in relation to their position at each time point. Cephalometric analysis revealed stability of Le Fort III midface advancement during follow-up. However, bone growth after midface advancement is limited, and as reported in the literature, almost no spontaneous growth is present because of the intrinsic nature of malformations. Patients treated in the growing age benefit from midface advancement, with resolution of exophthalmos, ocular bulb exposure, airway impairment, and good aesthetic outcome.

Bilateral orbital roof fracture.

Bilateral orbital roof fractures are rare events usually associated with high-energy impact trauma. The clinical picture is often multiple because of involvement of cranial, cerebral, and facial injuries. The primary diagnostic and therapeutic approaches aim to safeguard the cerebral state and to intercept the consequences of severe orbital trauma. The latter may present dramatic events and determine permanent ocular bulb or optic nerve damage, even vision impairment and blindness. Immediate intraorbital decompression decreases the pressure exerting directly or indirectly on the optic nerve. Surgical decompression of the orbit was performed in a young man showing almost complete blindness after bilateral orbital roof fracture. The final result showed good recovery of vision and functional motility of the bulbs.

Endoscopic endonasal versus transfacial approach for blowout fractures of the medial orbital wall.

AbstractIn the last decades, the introduction of computed tomography has allowed an increase in the number of diagnosed fractures of the medial orbital wall. To repair medial wall fractures, many surgical techniques have been proposed (1), each one with its advantages and disadvantages. In this study, we compared endoscopic endonasal and transcutaneous reduction approaches in terms of surgery time and clinical outcome. Between 2001 and 2005, 81 patients with orbital wall fractures were treated at our department. Among these 81 patients, 24 (29.63%) were affected by a medial orbital fracture. Patients with fracture to both floor and medial walls underwent floor reduction by a transcutaneous subpalpebral approach (n = 9, 11.1%), whereas patients with isolated medial wall fracture underwent medial wall reduction by a transcutaneous subpalpebral approach using alloplastic implants (n = 8, 9.88%) or were treated by endoscopic approach (n = 5, 6.17%). After surgery, oculomotor function improved in all 22 patients. None of the patients had complications. Computed tomography revealed a well-consolidated site of fracture in both endoscopic endonasal and transcutaneous approaches. The average operating time for endoscopic endonasal and transfacial approach was 50 and 45 minutes, respectively.In this paper, the author proposed a results comparison between the endoscopic approach and the transcutaneous one.

Three-dimensional temporomandibular joint modeling and animation.

The three-dimensional (3D) temporomandibular joint (TMJ) model derives from a study of the cranium by 3D virtual reality and mandibular function animation. The starting point of the project is high-fidelity digital acquisition of a human dry skull. The cooperation between the maxillofacial surgeon and the cartoonist enables the reconstruction of the fibroconnective components of the TMJ that are the keystone for comprehension of the anatomic and functional features of the mandible. The skeletal model is customized with the apposition of the temporomandibular ligament, the articular disk, the retrodiskal tissue, and the medial and the lateral ligament of the disk. The simulation of TMJ movement is the result of the integration of up-to-date data on the biomechanical restrictions. The 3D TMJ model is an easy-to-use application that may be run on a personal computer for the study of the TMJ and its biomechanics.

Juvenile idiopathic/rheumatoid arthritis and orthognatic surgery without mandibular osteotomies in the remittent phase.

Background Orthognathic treatment of patients with juvenile idiopathic/rheumatoid arthritis is still controversial. Mandibular procedures may result in relapse and further condylar resorption and pain. Maxillary osteotomies and genioplasty may be effective for the correction of malocclusion and for aesthetics. Patients and Methods Five patients with juvenile idiopathic/rheumatoid arthritis with severe temporomandibular joint (TMJ) involvement underwent orthognathic surgery with a simultaneous Le Fort I osteotomy and advancement genioplasty. The age of the patients at the time of surgery ranged between 17 and 29 years (mean, 21.75 years). The patients were under follow-up for a minimum of 8 months after surgery, and measurements were performed using Dolphin 3D imaging. Results All 5 patients have an improved occlusion and improved facial aesthetics. The mean mandible rotation advancement was 5.6 mm (range, 4 to 7 mm). The mean posteroanterior face height ratio (S-Go/N-Me) after surgery was 63.9 (range, 62.9 to 65.8). No exacerbation of the juvenile rheumatoid arthritis was encountered as a result of the surgical procedure. Conclusions Le Fort I osteotomy with impaction and advancement genioplasty are effective procedures for occlusal and aesthetic correction of juvenile idiopathic/rheumatoid patients. Mandibular procedures may evoke further condylar resorption with pain and functional impairment of the TMJ.

Lipostructure in Parry-Romberg disease.

Abstract Parry-Romberg syndrome is a disease characterized by progressive hemifacial atrophy. Multiple surgical procedures have been used to improve the facial volume and contours of patients with this disease, including alloplastic, silicone, or collagen implants; lipofilling; and pedicled or free-flap transplants. The present case describes the successful application of lipostructure to treat a woman with Parry-Romberg syndrome affecting the left side of her face.

Osteoradionecrosis of a mandible: A case report of implant-supported rehabilitation

The head and neck are the sixth most common sites of cancer in the world; the survival rate at 5 years from diagnosis is 60%. Surviving patients, after the critical phase of the disease, require proper rehabilitation. The treatment of oral neoplasia, such as surgery and radiotherapy, may often determine significant disability, such as impaired speech, swallowing, mastication and facial deformity, with severe consequences on the quality of life of these patients. Dental implant-based prosthodontic rehabilitation is a consolidated technique for improving the quality of life in patients who have overcome oral cancer. Implants provide stability and support for removable prostheses in oral cavities seriously deformed by surgical treatment. Moreover, mobile prostheses have the advantage of being removable, to check the health of oral tissues and intercept possible relapses of the neoplasia. On the other hand, a lack of residual bone following resection makes it difficult to place implants in an ideal position, and patients who have been submitted to radiotherapy of the head and neck are reported to have a reduced success rate. This paper presents the case of a 67-year-old woman rehabilitated with dental implant-based prosthesis after a hemimandibulectomy due to osteoradionecrosis, without bone reconstruction.

Metastatic Uterine Leiomyosarcoma in the Upper Buccal Gingiva Misdiagnosed as an Epulis

Uterine leiomyosarcoma (LMS) is a rare tumor constituting 1% of all uterine malignancies. This sarcoma demonstrates an aggressive growth pattern with an high rate of recurrence with hematologic dissemination; the most common sites are lung, liver, and peritoneal cavity, head and neck district being rarely interested. Only other four cases of metastasis in the oral cavity have been previously described. The treatment of choice is surgery and the use of adjuvant chemotherapy and radiation has limited impact on clinical outcome. In case of metastases, surgical excision can be performed considering extent of disease, number and type of distant lesions, disease free interval from the initial diagnosis to the time of metastases, and expected life span. We illustrate a case of uterine LMS metastasis in the upper buccal gingiva that occurred during chemotherapy in a 63-year-old woman that underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy for a diagnosis of LMS staged as pT2bN0 and that developed lung metastases eight months after primary treatment. Surgical excision of the oral mass (previously misdiagnosed as epulis at a dental center) and contemporary reconstruction with pedicled temporalis muscle flap was performed in order to improve quality of life. Even if resection was achieved in free margins, “local” relapse was observed 5 months after surgery.