Surgical management of syndromic versus non-syndromic craniofacial fibrous dysplasia: a systematic review and meta-analysis.

The main purpose of this study was to identify an algorithm for the surgical management of fibrous dysplasia in syndromic (McCune-Albright syndrome) and non-syndromic patients (monostotic and polyostotic subtypes). The secondary objectives were to assess the prevalence of affected craniofacial bones and the main clinical presentation. The authors performed a systematic review and meta-analysis by conducting a comprehensive electronic search from 1 January 2000 to 31 December 2019. A total of 1260 patients were included. The maxilla was the most affected facial bone (41%) (p<0.001, CI 38.3 to 43.8) and facial asymmetry was the chief complaint (p<0.001, CI 31.7 to 37.1). Conservative surgery registered higher recurrence rates than radical resection in both syndromic (84%) (p<0.001, CI 70.9 to 92.8) and non-syndromic patients (26%) (p<0.001, CI 21.8 to 30.6). Compared with prophylactic decompression, therapeutic optic nerve decompression (OND) showed better postoperative outcomes in both syndromic (p=0.9, CI 18.6 to 55.9) and non-syndromic patients (p=0.09, CI 9.3 to 28.4). Watchful waiting showed excellent results in both subgroups when asymptomatic (p<0.001). Syndromic and non-syndromic patients share the same treatment strategies. Radical resection is the preferred surgical technique to eradicate the disease, but it is often difficult to perform due to the extent and location of the disease. Furthermore, the authors advise early therapeutic over prophylactic OND to prevent optic nerve atrophy. Asymptomatic patients should be managed expectantly. Finally, medical management helps reduce the symptoms of bone pain (p=0.02 in non-syndromic and p<0.001 in syndromic patients).

Long-term evaluation of three-dimensional volumetric changes of augmented severely atrophic maxilla by anterior iliac crest bone grafting.

PURPOSE: The purpose of this study was to evaluate long-term three-dimensional graft resorption following reconstruction of the severely atrophic maxilla with anterior iliac crest bone grafting. METHODS: Twenty-two patients (13 males), who underwent autogenous bone grafting and implant placement to their severely atrophic maxillary alveolar ridges were identified and included in the study. Pre- and postoperative cone-beam computed tomography (CBCT) scans of 40 recipient grafting sites were evaluated to calculate volumetric changes over time. CBCT scans were performed preoperatively (V0) and one week (V1), three months (V2), one year (V3), and three years (V4) following the augmentation operation. RESULTS: The average graft resorption from V1 to V2, V1 to V3, and V1 to V4 was 31.42%, 33.96%, and 37.96%, respectively. Initial graft volume reduction within the first three months was statistically higher compared to other postoperative periods (P<0.013). The rate of resorption reduced slightly from the third month of the surgery (V2) (P>0.013). There was no statistical difference between resorption volume and gender, type of prosthesis, the presence of vestibuloplasty, or patient age (P>0.05). CONCLUSION: The overall success rate of the iliac bone block grafts was found to be high. The volumetric resorption rates associated with the graft were favourable for the reconstruction of the maxilla and for permitting the placement of dental implants three months after augmentation. The highest graft resorption was found at the third postoperative month. Placement and loading of the implants reduced the resorption rate slightly over time.

Oculovagal reflex in paediatric orbital floor fractures mimicking head injury.

BACKGROUND: The oculovagal reflex is well described in ophthalmic surgery, but may be caused by any manner of pressure on the globe. Children with orbital blowout fractures present in a different manner from adults. The classic presentation in children is a white eye injury (ie, no subconjunctival haemorrhage) with upgaze diplopia and general malaise. METHODS: A retrospective audit is presented of paediatric and young adult patients referred to the Orbital Service at St George's Hospital who required surgical intervention. RESULTS: One-third of children with orbital blowout fractures are admitted for head injury observations, while the true cause for the symptoms goes unrecognised and uninvestigated. CONCLUSIONS: Orbital blowout fractures in children require more swift intervention than in adults if muscle ischaemia and permanent impairment of the vision is to be avoided. The delay for head injury observation may therefore compromise the surgical outcome.

Association of corticosteroids and factor V, prothrombin, and MTHFR gene mutations with avascular osteonecrosis in renal allograft recipients.

The mechanism of posttransplantation avascular osteonecrosis (AVN) is controversial. Besides an increased bone marrow pressure due to reduced blood supply, enhanced coagulation has been considered. We investigated the associations of factor V Leiden, prothrombin G20210A, and MTHFR C677T mutations as well as cumulative corticosteroid doses with AVN in renal allograft recipients. The records of 39 volunteer patients and 11 patients in whom osteonecrosis was previously identified were reviewed for cumulative corticosteroid dosages during the first year. All patients were screened for factor V Leiden, prothrombin G20210A, and MTHFR C677T mutations by direct sequencing of genomic DNA. The cumulative corticosteroid dosages at 3, 6, and 12 months in the osteonecrotic group (5033.5 +/- 1565.3, 7164.9 +/- 2063.1, 8835.1 +/- 2216.8 mg) were significantly higher than in the control group (3629 +/- 1504.1, 4784.5 +/- 1568.7, 6322.4 +/- 1686.6 mg; P = .013, P = .001, P = .001, respectively). No significant difference in factor V Leiden, prothrombin G20210A, and MTHFR C677T mutations was observed between the osteonecrotic and control groups (P > .05). In conclusion, an association between the first year (3, 6, and 12 month) cumulative corticosteroid dosages and AVN was demonstrated in renal transplant recipients. However, no correlation was determined between factor V Leiden, prothrombin G20210A, and MTHFR C677T mutations and osteonecrosis.

Gemcitabine-induced vasculitis in advanced transitional cell carcinoma of the bladder.

BACKGROUND: Gemcitabine (GEM) is an alternative chemotherapeutic agent for patients with metastatic bladder cancer. It is believed to be a well-balanced agent, having acceptable toxicity and enhanced antitumor activity. The integration of GEM into the initial chemotherapy plan for these patients is still being developed. CASE REPORT: The patient, male, aged 56 years, was suffering from a transitional cell carcinoma of the bladder. Due to frequent local superficial recurrences, radical cystectomy with pelvic lymphadenectomy and continent ileal diversion was performed. Four years after the operation a left inguinal lymphadenopathy was noted and metastatic bladder carcinoma was confirmed on biopsy. Cytotoxic therapy combining GEM and cisplatin and local external irradiation therapy was initiated. The patient developed extensive necrotising vasculitis with muscle damage after the second course of therapy. Chemotherapy was stopped immediately but this was not enough to relieve the symptoms of severe myalgia and swelling, and additional treatment consisting of cyclophosphamide and prednisolone was initiated. CONCLUSION: Although GEM seems to be relatively safe, some unexpected complications may occur during treatment. This case is not common, but it reinforces the need for careful attention to any new symptoms that seem to be unassociated with the primary disease. Prompt evaluation of such symptoms should be carried out in patients receiving GEM therapy.

The prevalence of rheumatoid arthritis in an urban population of Izmir-Turkey.

OBJECTIVE: To estimate the prevalence of rheumatoid arthritis (RA) in an urban area in Izmir, Turkey. METHODS: The study was conducted in the Balcova and Narlidere districts of Izmir and a total of 2,887 people aged 20 years or older were contacted with a 98.2% acceptance rate. Nine medical doctors administered an RA questionnaire by face-to-face interview. Subjects reporting a history of swelling in at least 2 joints lasting more than 4 continuous weeks or a history of a diagnosis of rheumatoid arthritis, inflammatory joint rheumatism or joint rheumatism were considered as screening positive and they were invited to come in for an examination. RA cases were defined by the 1987 American College of Rheumatology (ACR) criteria modifiedfor use in population studies. RESULTS: A total of 301 subjects (243 women, 58 men), or 10.6% of those who received the questionnaire were screening positive. 240 (79.7%) of these agreed to undergo a clinical examination either in the clinic or at home. Among these, 14 (12 female, 2 male) patients fulfilled the ACR criteria for RA. The prevalence of RA was 0.49% (95% CI 0.27-0.83) in the total population interviewed, 0.77% (95% CI 0.40-1.35) in women and 0.15% (95%CI 0.02-0.60) in men. The age- and sex-adjusted prevalence for the general population was estimated as 0.36%. Five of the 14 RA (36%) cases had not been diagnosed previously CONCLUSION: These data are consistent with the results of other Mediterranean countries. A significant proportion of RA cases remain undiagnosed in the community.

Giant thrombosed aneurysm of the vertebral artery in childhood: an extremely rare case.

In childhood, a significant proportion of giant aneurysms usually occur at the vertebrobasilary system. Nonetheless, giant totally thrombosed aneurysm which only involves the vertebral portion of the vertebrobasilary system is very rare. Up to this time, in childhood, a few giant aneurysm originating from vertebral artery have been reported, and to our knowledge only one case was reported to be totally thrombosed. The enlargement tendency of totally thrombosed aneurysms and management of these cases are still controversial. Thus, diagnosis and follow-up of cases where a radical approach could not be performed require a multi-modality radiologic approach. In this study, we reported a case of angiographically occult giant thrombosed vertebral artery aneurysm, which is extremely rare, and discussed the importance of CT and MRI in evaluation of these patients before surgical treatment.

Retromandibular approach to the mandibular condyle: a clinical and cadaveric study.

This prospective study was carried out to assess the morbidity of the retromandibular approach in the management of condylar fractures. Twenty consecutive patients underwent open reduction and internal fixation of their condylar fractures using this technique. Branches of the facial nerve were encountered in six cases (30%). Temporary weakness of the facial nerve occurred in six patients (30%), but this resolved in all cases within 3 months and there were no cases of permanent nerve injury. Two patients had a temporary deficit of the great auricular nerve and one patient developed a sialocoele that resolved with aspiration. A cadaveric study using 30 facial halves (15 fresh cadavers) was also conducted. Branches of the facial nerve were encountered in 12 dissections (40%). The literature regarding facial nerve morbidity in relation to the management of condylar fractures is reviewed.

Ocular psychiatric disorders in maxillofacial injuries. Two case reports and a review of psychogenic ocular symptoms.

Ocular signs and symptoms associated with facial trauma can be a manifestation of an underlying psychiatric disorder which may exist prior to the traumatic incident, or even develop as a result of the stressful traumatic experience in a psychologically vulnerable individual. Two such cases are presented. A brief review of psychogenic ocular symptoms as well as suggestions for evaluation and treatment are discussed.

Pigmented villonodular synovitis of the knee presenting as a popliteal cyst.

Pigmented villonodular synovitis is a locally aggressive tumor of the synovium of joints and tendon sheaths. It is commonly seen in the synovial lining of the flexor tendons of the hand and in the synovium of the knee and less commonly in other joints. A case of pigmented villonodular synovitis of the knee in a 60-year-old man, with an intra-articular origin extending extra-articularly, is presented. The interesting point is that the initial diagnosis was a Baker cyst.

A multicentre study of abdominal aorta diameters in a Turkish population.

The aim of this study was to determine the normal values for aortic diameters and the prevalence of aortic dilatation in a mixed Turkish population. Between March 1998 and May 2000, patients who were undergoing abdominal ultrasonography examination for pathologies not involving the aorta, in three different cities, were enrolled into the study prospectively. The anterior posterior aortic diameters were measured at the subdiaphragmatic and aortic bifurcation levels using ultrasonography. A total of 596 patients were included (302 females, 294 males). The mean age was 48 +/- 16 years (range, 6-88 years). The mean aortic diameter in the whole group was 19.0 +/- 3.9 mm (10-45 mm) at the subdiaphragmatic level and 15.7 +/- 3.6 mm (9-65 mm) at the aortic bifurcation level. The mean subdiaphragmatic aortic diameter was 18 +/- 3 mm in females and 19 +/- 4 mm in males. The mean aortic diameters at the bifurcation level was 15 +/- 3 mm in females and 16 +/- 4 mm in males. An aortic bifurcation diameter > 30 mm was encountered in 0.67% of the population. This ratio increased to 1.8% in patients over 55 years of age, regardless of sex. A subdiaphragmatic aorta diameter above 30 mm was observed in 1.2% of the population. In patients over the age of 55 years, this ratio increased to 2.7% (3.6% in males and 1.9% in females). In this national study, the subdiaphragmatic aortic diameters were similar to mean values reported in the world literature. The mean aortic bifurcation diameters were generally lower when compared with the literature, which may be due to difficulties in standardization of the measurements. Aneurysmal dilatation rates in this study also conform to those reported in studies conducted in other countries. Considering the significant number of patients with aneurysmal dilatation of the aorta in the elderly population, we believe it would be prudent to evaluate the aorta in all patients undergoing abdominal ultrasonographic examination.

Adenomatoid hyperplasia of palatal minor salivary glands.

Adenomatoid hyperplasia of palatal minor mucous glands is rare but significant because the clinical appearance mimics malignant disease. The typical history of a painless, indolent palatal swelling, together with the histological picture of benign glandular hyperplasia and hypertrophy, are illustrated in this report.

The role of open reduction and internal fixation in unilateral fractures of the mandibular condyle: a prospective study.

The purpose of this report is to present our experience in the management of unilateral condylar fractures between 1995 and 1998. This prospective study was carried out at a Regional Maxillofacial Unit and Teaching Hospital in the UK, and included 54 patients, of whom 32 attended for review. In all, 42 men and 12 women (age range 17-40 years) entered the study: 28 patients had unilateral fractures of the mandibular condyle alone; the remainder also had another mandibular fracture, invariably at the parasymphysis. Thirty-five of the patients (65%) had allegedly been assaulted. This is at variance with a recent study published by the British Association of Oral and Maxillofacial Surgeons, which showed that only 24% of facial injuries were the result of inter-personal violence. Open reduction and internal fixation of the mandibular condyle seems to carry low morbidity and may be of considerable functional benefit to the patient.

Dysmorphophobia: recent developments of interest to the maxillofacial surgeon.

Recent changes in the classification of psychiatric illnesses have resulted in the term dysmorphophobia being replaced by that of body dysmorphic disorder (BDD). This paper attempts to alert the clinician to the presenting features of the condition and discusses its management, with particular emphasis on the role of surgery and current concepts of pharmacological treatment. A number of case reports are included to illustrate the diversity of this interesting disorder and the difficulties involved in treatment.

Cranio-orbital primary intraosseous haemangioma.

PURPOSE: Primary intraosseous haemangioma (IOH) is a rare benign neoplasm presenting in the fourth and fifth decades of life. The spine and skull are the most commonly involved, orbital involvement is extremely rare. We describe six patients with cranio-orbital IOH, the largest case series to date. PATIENTS AND METHODS: Retrospective review of six patients with histologically confirmed primary IOH involving the orbit. Clinical characteristics, imaging features, approach to management, and histopathological findings are described. RESULTS: Five patients were male with a median age of 56. Pain and diplopia were the most common presenting features. A characteristic 'honeycomb' pattern on CT imaging was demonstrated in three of the cases. Complete surgical excision was performed in all cases with presurgical embolisation carried out in one case. In all the cases, histological studies identified cavernous vascular spaces within the bony tissue. These channels were lined by single layer of cytologically normal endothelial cells. DISCUSSION: IOCH of the cranio-orbital region is rare; in the absence of typical imaging features, the differential diagnosis includes chondroma, chondrosarcoma, bony metastasis, and lymphoma. Surgical excision may be necessary to exclude more sinister pathology. Intraoperative haemorrhage can be severe and may be reduced by preoperative embolisation.

An unusual presentation of a maxillary central giant cell granuloma.

Central Giant Cell Granulomas (CGCG) of the jaw are uncommon benign lesions which usually occur in the mandible in young adults. Here we present a case report of a 45-year-old woman who presented with a 5-month history of right nasal obstruction secondary to a large maxillary CGCG. The age, symptoms at presentation, location and radiographic features were not pathognomonic for this lesion. This case helps to demonstrate the wide variation in the clinical, radiological and histopathological features of CGCC and provides an update in current treatment concepts.

Resorption of a sequestered cervical disc confirmed by magnetic resonance imaging: long term follow-up. Case report.

In literature cervical disc extrusions are considered by most neurosurgeons a definitive indication for surgery. This approach may stem from a fear of disc fragment migration with neurological deterioration. We report a rare case of cervical disc sequestration with a seldom prognosis which resolved spontaneously in a two-month follow-up on magnetic resonance imaging (MRI), emphasizing the efficacy and applicability of conservative treatment in cervical disc herniations. Even with the basic conservative treatment methods we observed the prominent decrease in symptoms and spontaneous total resorption of the sequestered fragment. Conservative treatment in sequestered cervical discs with no neurological deficit can be an alternative therapeutic approach with the guidance of MRI.

Tumor grade-related thallium-201 uptake in chondrosarcomas.

OBJECTIVES: Diagnosis of low-grade chondrosarcoma, especially discrimination between enchondroma and low-grade chondrosarcoma, may be difficult pathologically. The aim of this study was to evaluate the value of thallium-201 (Tl-201) scintigraphy in the diagnosis of chondrosarcoma and to investigate whether there was a correlation between Tl-201 uptake and tumor grade. METHODS: We retrospectively evaluated 121 patients with pathologically proven bone and soft tissue tumors diagnosed between the years 1999 and 2007. All patients were followed by the Bone and Soft Tissue Tumor Working Group in our hospital. Twenty-three patients, mean age 44 +/- 15 (range 17-72) years, with a diagnosis of cartilaginous tumors were included. Increased Tl-201 uptake at the lesion sites greater than background was evaluated as malignant tumor. For the pathologic classification, a grading system (grade 1-3) based on the histopathologic findings was used. Pearson correlation coefficient was used to determine whether there was any correlation between Tl-201 uptake and tumor grade in chondrosarcoma. RESULTS: There were 7 enchondromas and 16 chondrosarcomas. Four of 16 patients with chondrosarcoma had lesions pathologically classified as grade 3, 5 as grade 2, and 7 had grade 1 chondrosarcoma. Increased Tl-201 uptake was observed in all patients with grade 3 chondrosarcoma and 2 patients with grade 2 chondrosarcoma. Of 10 patients with chondrosarcoma, 3 grade 2 chondrosarcomas and 7 grade 1 chondrosarcomas, there was no Tl-201 uptake in the tumor region. A significant correlation was found between Tl-201 uptake and tumor grade in chondrosarcoma (p = 0.002, r = 0.71). Only a few reports in literature have demonstrated false negative results in low-grade chondrosarcoma. CONCLUSION: Tl-201 uptake was related to tumor grade in chondrosarcoma. If there is a possibility of chondrosarcoma, Tl-201 scintigraphy should be reported with caution.

Gorham disease: correlation of MR findings with histopathologic changes.

Gorham disease is a rare disorder of unknown etiology characterized by bone destruction and abnormal proliferation of thin-walled vascular channels including lymphatic capillaries. Starting monocentrically in a single bone, the angiomatous masses in this disease extend to adjacent bones and soft tissues without respecting articular barriers. Herein we report a case of Gorham disease with its MR and histopathologic appearance.