Gianluigi Longobardi

Fanconi anemia manifesting as a squamous cell carcinoma of the hard palate: a case report

Fanconi Anemia is a rare autosomal recessive disorder characterized by various congenital malformations, progressive bone marrow failure at a very young age and of solid tumors development. The authors present a rare case of a squamous cell carcinoma of the hard palate in a Fanconi Anaemia patient. The atypical clinical manifestation rendered the diagnosis more difficult. This case, for age of appearance, sex and localization, is unique in international literature. We recommend a quarterly follow up of the oral-rhino-pharynx complex in FA patients and to consider as carcinomas, all oral lesions that last more than two weeks.

Hamartomatous sialolipoma of the submandibular gland: case report.

Sialolipoma is a rare tumour of the salivary gland that is composed of mature adipocytes and normal salivary gland tissue. We report an unusual case of a sialolipoma of the submandibular gland in a 77-year-old woman. The location of this tumour has not to our knowledge been previously described. The tumour was excised and has not recurred during 22 months postoperatively.

Surgical Therapy for Osteonecotic Lesions of the Jaws in Patients in Therapy With Bisphosphonates

Objective: Surgical therapy to improve the symptoms and the lesions in osteonecrosis (ON) of the jaws in patients in therapy with bisphosphonates. Design: to evaluate the patients therapeutic protocols, performance status, and factors promoting ON to prevent surgical failure. Materials and Methods: 18 patients affected by osteonecrotic lesions of the jaws associated to BF, were treated by surgery. Results: The results were recorded after 6 months. All the patients showed improvement of symptoms, in particular the pain. In addition, all the patients referred to a sensation of fresh and clean mouth, the disappearance of fetor ex ore, and a healthy mouth. Conclusions: The management and the resolution of BF osteonecrotic lesions is arguable and complex because in most cases, the patients are affected by oncologic disease when the better approach is prevention, but when the ON lesion is clear, surgery can improve the symptoms and in some cases, it can be resolute. To prevent surgical failure, it can be useful to evaluate the patients therapeutic protocols, performance status, and factors promoting ON.

Le Fort I osteotomy and the use of bilateral bichat bulla adipose flap: an effective new technique for reconstructing oronasal communications due to cocaine abuse

The aim of the study was to describe a new and effective method for reconstructing small- and medium-sized oronasal communications in cases of deficient blood supply of oronasal mucosa. A male patient, aged 45, was presented with a hard palate defect due to chronic cocaine inhalation. The defect was corrected using bilateral Bichat bulla adipose flap and a Le Fort I osteotomy. The surgical technique was described, together with its advantages. Surgery lasted 2 hours. The reconstructive technique had been easy to execute. Six months after the surgery, the defect has been corrected without complications. Le Fort I osteotomy and the use of a bilateral Bichat bulla adipose flap is an effective technique to correct small- and medium-sized palatal defects not solvable with simple oral mucosa flaps. The technique is easy to execute and it showed a high efficacy with minimal impact on the patients esthetic appearance.

A new method for assessment of craniofacial malformations.

Many assessments of craniofacial malformations are generally undertaken to assist in surgical intervention including physical examination, cephalometric radiographs in anteroposterior and lateral views, stereolithographic models, and anthropometric measurements integrated with three-dimensional computed tomography (3-D CT) reconstructions to quantify skeletal deformities. In the present report, the use of 3-D Malformation Analysis, a three-dimensional methodology for planning craniofacial operative procedures, is presented. In addition to cephalometric and anthropometric databases, the measurements from 3-D surface reconstructions from CT were used intraoperatively to establish the correct position of skeletal segments.

Follicular cyst of the jaw developing into a keratocyst in a patient with unrecognized Gorlin-Goltz syndrome.

Gorlin-Goltz (GG) syndrome is an inherited autosomal dominant condition. Its diagnosis may be clinically confirmed by checking either major or minor signs that define the diagnostic criteria. It may occur that, although GG syndrome is a well-known condition, only the specific symptom could be observed by different specialists. Therefore, the patient cannot be placed into an always complex clinical panel. We introduce an example in this report. Throughout a 20-year clinical history characterized by the lack of proper diagnosis and missed follow-up operations, a patient with GG syndrome underwent partial amputation of the jaw after severe complications. A 52-year-old man required an implant-prosthetic rehabilitation since becoming edentulous after a partial resection of the jaw due to a keratocyst, which was later reconstructed through a free fibula flap. The observation of a typical phenotype and various symptoms that succeeded for longer than 20 years, with anamnestic evaluation and clinical examination, led us to suspect a complex pathologic condition such as GG syndrome, which was not previously considered, although the patient had undergone several polyspecialistic evaluations. Diagnosis has been eventually confirmed by a genetic study, which was always mandatory. The simultaneous presence of muscular and skeletal malformations, basocellular nevi, and multiple cysts of the jaw can represent signs linking to a condition such as GG syndrome. There are many syndromes involving the head and neck region, and specialists are supposed to be alerted when faced with similar typical expressions associated with a characteristic soma so as to avoid delays in diagnosing the syndrome.

Orthognathic surgery: an informed consent model.

In this study, the authors propose an informed consent form for orthognathic surgery. A careful review of the international literature and clinical practice suggested the feasibility of dividing the informed consent form into two parts. In first part, the diagnostic procedures and the therapeutic and surgical times are described. The patient must sign it as soon he or she accepts the orthodontic/surgical therapy proposed. The second part describes the possible problems and difficulties of the procedure, and it must be read by the patient before starting the therapy. In the opinion of the authors, this informed consent form allows the patient to know the risks related to the orthodontic/surgical therapy, thus preserving the surgeons from the civil risk and penalties of omission.

Rhytidectomy Associated With Autologous Fat Transplantation in Parry-romberg Syndrome

Progressive hemifacial atrophy or Parry-Romberg syndrome is an uncommon degenerative and poorly understood condition characterized by progressive atrophy of 1 side of the face. It may involve several layers of tissue manifesting itself in a more or less aggressive form (mild, moderate, and severe). Generally, the restoration of contour and symmetry are the goals of the therapy in patients affected by this syndrome. In this article, we present the technique and the 6-year postsurgery result of a case of Parry-Romberg syndrome treated with 1-stage anterior lifting, removal of superficial muscular aponeurotic system, and autologous fat transplantation because the patient requested to recover more than atrophy and also 20 years of lost youth.

A new 3-phase therapy protocol in temporomandibular joint ankylosis: our experience

Limitations in oral opening may be due either to intra-articular or extra-articular ankylosis (pseudoankylosis). The principal means of therapy usually consist of 2 steps: surgical removal of the ankylotic block and immediate functional rehabilitation. In the postoperative period, however, immediate and adequate functional rehabilitation is not always possible because of pain and swelling of the temporomandibular joint, resulting in a very high risk of relapse. To prevent this, the authors introduce a third phase between the 2 already mentioned (surgery and functional therapy) in which the oral opening obtained with surgery is held in place while clinical conditions (pain and swelling) are allowed to stabilize before initiating intensive physiotherapy. Through the discussion of 18 clinical cases (8 of which were treated in the traditional way), the authors report on the intermediate treatment phase introduced by them between the surgical stage and the rehabilitation. Immediately after the operation, a handmade wedge, that is, a bite block of suitable size, is placed between the dental arches for a period of about 3 weeks, while appropriate pharmacological therapy was administered. The results showed an improvement of the mandibular movements both in opening and laterally, on the right and left sides, in patients treated with the new protocol. The proposed protocol holds the opening obtained with surgery until the clinical conditions make it possible to initiate intensive physiotherapy. In this way, the authors obtained better results in both recovery and in mandibular movements.

Mesentery-like steri-strip: a scarless drain fixation.

Dear Editors, Recently in your journal an interesting trick in steri-strips application was suggested by D’Ettorre et al. (1), we would like to draw the readers’ attention to a further potential use of this common dressing. In fact, suction drains are usually secured by looping suture material around the drain in the form of a ‘Roman garter’. However, the knot is prone to loosening, which allows the drain to slip and the vacuum that creates the suction effect to be lost (2). To avoid slippage, silk is used to create sufficient friction around the drain such that it is secured in place, but excessive force can cause blockage of the tube and clotting. Furthermore, the knot can be the source of contamination (3) or highly irritant (4) to skin and can cause local necrosis if the knot is strongly tightened (2). Various methods have been proposed to overcome these problems (5, 6), but they all require sutures to be inserted, creating permanent signs on the skin. In most surgical procedures the drainage is removed postoperatively within the first 24 hours. Hence, strong attachments to enable prolonged fluid drainage become useless. In order to minimise the signs of scarring, we want to draw your readers’ attention to our method for drain fixation with steri-strips after surgery. The skin is prepared for the insertion of drainage by rinsing and drying. The drain is placed in the desired position and fixed to the skin with the use of multiple steri-strips, which are shaped to create a ‘mesentery’. This mesentery arises 1–2 cm distal to the point where the drain left the skin (Figure 1). It is then further secured by other steri-strips placed perpendicular to the first ones. Finally, Mastisol® Adhesive liquid is spread over the steri-strips to ensure the maximal holding power. Our simple method for securing drains avoids the use of sutures and is of low cost per laceration and infected laceration compared with sutures (7). It is quick and as long as the steri-strips are used for wound coverage and suture fixation, no extra costs are required. It is very useful in situations where drains need to be in situ for a short period. It is simple to