Dental anomalies in pediatric patients with familial adenomatous polyposis.

Familial adenomatous polyposis patients often present with non-malignant extra-intestinal manifestations which include dental anomalies that may be evident prior to the appearance of the colonic adenomas. The aims of this study were to describe the prevalence and type of dental anomalies and the relationships between gene mutations and dental anomalies in these patients. Twenty-two pediatric familial adenomatous polyposis patients and 46 controls, who were age and gender matched participated. Familial adenomatous polyposis patient's had a dental examination with panoramic radiograph and medical record review for age at diagnosis, the presence of the adenomatous polyposis coli gene mutation, and determination of other extra-intestinal manifestations on the body. The control group was identified from a retrospective chart review and selected if there was a current panoramic radiograph. The only significant difference between familial adenomatous polyposis patients and controls were the presence of jaw osteomas and sclerosis (p = .0001). Patients with a mutation in, or upstream of codon 1309 had a higher frequency of osteomas (77.8%) and jaw-bone sclerosis (44.4%), and 77% of these had at least one dental anomaly. This preliminary study showed an association between a genetic variant at, or upstream of codon 1309, and radiographic dental anomalies.

Types of orbital osteoma - A descriptive analysis.

AIMS: This study describes different types of orbital osteoma based on clinical per-operative morphology and radiological findings to facilitate communication between ophthalmologists and surgical management for a better patient outcome. MATERIALS AND METHODS: The study was conducted in the Orbit and Oculoplastics Department of Al-Shifa Trust Eye Hospital, Rawalpindi, Pakistan, from July 1, 2001 to June 30, 2014. A retrospective analysis of 520 diagnosed orbital tumours who presented to us was carried out and the prevalence of orbital osteoma was determined. The sampling technique was non-randomized sampling. Based on the clinical morphology observed during surgical intervention as well as the radiological findings of the orbital osteoma, a simple yet useful description of orbital osteoma was introduced. RESULTS: The retrospective analysis of 520 diagnosed orbital tumours revealed a prevalence of 2.3% (n = 12) of orbital osteoma. Based on our observation of the 12 (n = 12) cases of orbital osteoma, we have classified orbital osteoma into a combination of seven types: "sessile" or broad-based osteoma; "pedunculated" or mushroom osteoma with a thin cylindrical stalk of origin; "dumb-bell"-shaped osteoma with the simultaneous presence in orbital and adjoining sinus/nasal cavity; "wrapped" osteoma, covered by a thin cartilaginous layer; "naked" osteoma, without any such covering; "homogenous" or uniformly dense osteoma; and "heterogeneous", soft-dense osteoma with varying areas of density. CONCLUSION: This study attempts to introduce a simple description of different types of orbital osteoma based on clinical per-operative morphology and radiological findings for the first time to facilitate the surgical removal of orbital osteoma.

Ostéomes maxillo-mandibulaires : épidémiologie, diagnostic, classification et thérapeutique au centre hospitalier et universitaire de Yaoundé (Cameroun)

Cette étude rétrospective a porté sur les ostéomes, sur une durée de quatre ans au CHU de Yaoundé. En tenant compte des données cliniques et des comptes rendus opératoires, les patients dont les dossiers jugés satisfaisants pour remplir les critères d'inclusions ont été retenus.Il est apparu que les ostéomes sont peu fréquents dans notre contexte puisqu'ils ne représentent que 12,6 % des tumeurs osseuses. Ils se développent lentement et sans douleurs aux maxillaires comme à la mandibule. Cependant ils se localisent préférentiellement aux maxillaires. Ces ostéomes sont plus fréquents chez la femme et touchent surtout les adultes jeunes. Les diagnostics cliniques, radiologiques et histologiques sont assez aisés, permettant de classer les ostéomes en exostose et en énostose (endostose) selon qu'ils ont une évolution extra ou endosseuse. Le traitement est uniquement chirurgical et ne donne pas lieu aux récidives

Characteristics of paranasal sinus osteoma and treatment outcomes.

CONCLUSION: The incidence of paranasal sinus (PNS) osteoma was 6.4%. The most common site of PNS osteoma was the ethmoid sinus. All surgically treated patients underwent endoscopic surgery, and there was no recurrence in any patient. Technical improvements, including an image guidance system, extended the indications for endoscopic surgery for PNS osteomas, especially in the frontal sinus region. OBJECTIVE: The purpose of this study was to investigate the incidence and location of PNS osteomas detected by computed tomography (CT) scan at our hospital, and to describe our experience in the surgical treatment of PNS osteomas. METHODS: This study was performed on 1724 patients undergoing CT scans because of suspected sinus disease between 2004 and 2013. Endoscopic surgery was performed in 34 symptomatic patients. Medical records of the patients were reviewed, and clinical findings and treatment outcomes were investigated. RESULTS: PNS osteomas were detected in 110 patients (6.4%). Triple osteomas were detected in two patients. Double osteomas were detected in seven patients. In total, 121 lesions were identified as PNS osteomas. The ethmoid sinus was the most commonly affected site (57.0%), followed by the frontal sinus (25.6%), frontal recess (9.1%), maxillary sinus (5.0%), olfactory fissure (1.7%), and sphenoid sinus (1.7%) in descending order of frequency. Thirty-three patients were surgically treated for PNS osteomas through a purely endoscopic approach, and one patient with a frontal sinus osteoma underwent combined endoscopic surgery and frontal trephination. Image-guided surgery was performed in nine patients with involvement of the orbit and skull base, including the frontal sinus/recess. There were no major surgical complications and there was no tumor recurrence.

Review of 43 osteomas of the craniomaxillofacial region.

PURPOSE: To present and discuss the demographic and clinical aspects and the management of 44 cases of osteomas of the craniomaxillofacial region. MATERIALS AND METHODS: A retrospective chart review was performed of all cases of osteoma diagnosed from 2000 through 2010. The data collected included age at diagnosis, gender, lesion location, presenting symptoms, type of osteoma, treatment, and outcomes. RESULTS: Forty-two patients with 43 osteomas were diagnosed during the study period. Their mean age was 48 years. The male-to-female ratio was 0.4:1. Twenty-one patients were asymptomatic, whereas 10 patients complained about headache and neuralgia, and 11 patients presented with facial asymmetry. Only 21 symptomatic osteomas were surgically removed after histologic diagnosis, whereas for the asymptomatic lesions a careful follow-up was maintained. CONCLUSIONS: The slow growth of osteomas allows a conservative attitude toward asymptomatic lesions. Thus, when surgery is performed, it is extremely important to plan a surgical approach that minimizes any damage to the adjacent structures.

[Primary bone tumors of the orbit]. / Tumeurs primitives des parois osseuses orbitaires.

Primary orbital bone tumors account for 0.6-2% of all orbital tumors. This is a heterogeneous group of tumors arising from osseous, cartilaginous, fibrous, and vascular tissues. The two most commonly encountered lesions are fibrous dysplasia and osteoma. Treatment of the primitive orbital bone tumors is generally surgical.

A prospective study of paranasal sinus osteomas in 1,889 cases: changing patterns of localization.

OBJECTIVES/HYPOTHESIS: We developed the largest paranasal sinus computed tomography (CT) scan study so far by including 1,889 cases to investigate the prevalence, localization, age distribution, and the secondary complications of paranasal sinus osteomas. STUDY DESIGN: Prospective study. METHODS: A prospective study was performed on 1,889 consecutive adult individuals who underwent paranasal sinus CT examinations with suspected sinus disease. RESULTS: The prevalence of paranasal sinus osteomas was determined to be 3%. Osteomas were located most frequently in the ethmoid sinuses. The size of the osteomas varied from approximately from 2 to 30 mm. Of the osteomas found, 37%were accompanied by pathological sinonasal findings. CONCLUSIONS: The prevalence of sinonasal osteoma in the paranasal sinus region detected by CT scan is supporting the literature, whereas the localization pattern challenges it.

[Tumours of the bony face in children the treated at the Paediatric ENT Clinic in Warsaw]. / Guzy twarzoczaszki u dzieci leczonych w Klinice Otolarynologii Dzieciecej w Warszawie.

INTRODUCTION: The tumours of bony face at in children are rare. Among the most commonly found are inflammatory tumours and development anomalies. In histopathology, the most frequent fund are neuroma, fibromas, osteoma, lipomas. In inflammatory tumours there are abscess and inflammatory infiltration. Malignants tumours in this area are rare in children, but are mainly sarcomata or melanoma. Developmental tumours include cyst and fistula. OBJECTIVE: To analyse tumours of the bony face treated in Paediatric ENT Clinic in Warsaw. MATERIAL: There were thirty cases of bony face tumours treated in Paediatric ENT Clinic in Warsaw between 2005 and 2007. RESULTS: One cases (3%) provedto be malignant, and one (3%) locally malignant. Twenty-one cases (70%) were found to be developmental tumours, three cases (10%) of abscesses, and four cases (13%) of benign tumours. CONCLUSIONS: The most frequently-found tumours of bony face in children are developmental tumours. All tumours must be the subject of histopatological examination. In the case of cancers tumours it has to be established if the tumour is a primary or a metastatic. In the group analysed group the rare tumour were epithelioma, ganglioma.

[Endoscopic removal of sinonasal tumors]. / Endoskopik sinonazal tümör rezeksiyonu.

OBJECTIVES: To evaluate the efficacy of endoscopic surgery for sinonasal tumors. PATIENTS AND METHODS: Clinical records of 20 patients (10 males, 10 females; mean age 34; range 12 to 63 years) who underwent endoscopic tumor resection with diagnosis of sinonasal tumor between January 1998 and August 2004 were retrospectively reviewed. Patients who were operated by transnasal endoscopic surgery or by combined (endoscopic or external) surgical methods were included in the study. Patients were reviewed for age, sex, pathologic diagnosis and tumor location, surgical techniques, need for additional surgery, complications, recurrence, and follow-up period. RESULTS: There were five osteomas, one fibrous dysplasia, one ossifying fibroma, seven inverted papillomas, one oncocytic papilloma, three angiofibromas, one schwannoma, and one esthesioneuroblastoma. The patients follow-up period ranged between 6 months and 6 years (mean 26 months). The fibrous dysplasia involving the posterior ethmoid sinuses was subtotally resected due to its close proximity to the optic nerve. One inverted papilloma of the maxillary sinus was resected by a combined endoscopic and Caldwell-Luc approach. A combined endoscopic intranasal and external frontoethmoidectomy approach was performed for the removal of frontal sinus schwannoma. Recurrence was observed in one of the inverted papilloma cases. Total removal of the tumors was achieved in all other cases without any complications or recurrences. CONCLUSION: Low recurrence rates in our series showed that endoscopic resection of nasal-paranasal sinus tumors in selected cases, may be an appropriate method as solely or in combined with other surgical techniques for experienced surgeons.

Osteoma gigante mastoideo / No disponible

Los osteomas de mastoides son tumores óseos benignos de crecimiento lento y escasa frecuencia. Habitualmente son asintomáticos, siendo la deformidad estética su única manifestación y la causa de la indicación quirúrgica. Presentamos el caso clínico de un varón que presentabauna gran tumoración retroauricular y otra frontal de pequeño tamaño compatibles con el diagnóstico de osteoma

The osteomas of the mastoids are slow growing benign bone tumors of infrecuent occurance. Usually these tumors are asymptomatic, with cosmetic deformities as the only manifestation and cause for surgery. In this clinical case, we present a man with a large retroauricular tumor and a small frontal tumor. Both of the man's tumors are compatible with the diagnosis of osteoma

Qualitative diagnosis of calvarial metastasis by neural network and logistic regression.

RATIONALE AND OBJECTIVES: To simplify the diagnostic features used by an artificial neural network compared with logistic regression (LR) in the diagnosis of calvarial metastasis with computed tomography and analyze their accuracy. MATERIALS AND METHODS: Twenty-one of 167 patients with calvarial lesions were found to have metastasis. Clinical and computed tomography data were used for LR and neural network models. Both models were tested with the leave-one-out method. The final results of each model were compared using the area under receiver operating characteristic curve (Az). RESULTS: The neural network identified metastasis significantly more successfully than LR with an Az of 0.9324 +/- 0.0386 versus 0.9192 +/- 0.0373, P = .01. The most important features selected by the LR and neural network were age and edge definition. CONCLUSION: Neural networks offer wide possibilities over statistics for the study of calvarial metastases other than their minimum clinical and radiologic features for diagnosis.

Choroidal osteoma in Oriental patients.

BACKGROUND: Choroidal osteoma is a rare tumour of the choroid. This is the first report of cases of choroidal osteoma in Thai patients. OBJECTIVE: To report the clinical characteristics, imaging findings and long-term follow-up of choroidal osteoma in four Oriental patients. METHOD: Four cases of choroidal osteoma were observed for 5 years or more. RESULTS: All patients were young female patients whose ages ranged from 24 to 37 years. Three were unilateral and one was bilateral. The tumors were located at the juxtapapillary and macular area with overlying serous retinal detachment. Two patients had previous thyroid diseases and one was pregnant when the tumors were diagnosed. Osteoma did not develop in the vicinity of posterior staphyloma of high myopic eyes. Echography showed acoustic features of a plano-convex sonically dense lesion with high reflectivity echoes which persisted despite lower system sensitivity. None had subretinal neovascularization. Subretinal fluid disappeared spontaneously within one to 14 months in three patients. Gradual growth of the tumor in a pseudopodium manner developed from two to six years after initial examination. Decalcification occurred spontaneously or after laser ablation. CONCLUSIONS: The authors presented four Oriental patients with choroidal osteoma who were observed for at least 5 years. Echography is the best method for identifying this lesion and has unique acoustic features. Subretinal fluid can be seen in the absence of subretinal neovascularization and resorbs spontaneously. Decalcification occurred as a natural process or after laser ablative treatment. Hormonal changes may implicate the development of this tumor.

[Osteomatous jaw lesions in familial adenomatous polyposis]. / Osteomas mandibulares en la poliposis adenomatosa familiar.

The first aim of this study is to assess the frequency of osteomatous jaw lesions in patients with a diagnosis of familial adenomatous polyposis (FAP), and a group of FAP-relatives from the FAP registry of Majorca (Balearic Islands, Spain). The second aim is to study the predictive significance of osteomatous jaw lesions in families with FAP. The study included forty-two people. Twenty-five patients and seventeen relatives. All individuals underwent pantomography. Osteomatous jaw lesions were diagnosed in 68% of FAP-patients, and 6% of FAP-relatives. The difference was statistically significant (p < 0.001). The sensitivity was 68%, the specificity 94% and positive predictive value 94%. This frequency of osteomatous jaw lesions agree with previous studies of FAP-patients (57-82%), but differ from these of the previous studies of FAP-relatives (16-18%). The available method detect APC (adenomatous polyposis coli) gene mutations in 48-82% of FAP families. In the families without detected mutation, non-informative study or non-available study, the screening is based in seriated sigmoidoscopy and study of the extraintestinals lesions of the FAP syndrome. The frequency of osteomatous jaw lesions in FAP-patients is greater than the observed in FAP-relatives (p < 0.001). Osteomas in families with FAP are of predictive significance.

Osteoma miliar de la cara / Multiple miliary osteoma of the face

Se trata de una mujer de 40 años, sin antecedentes de acné juvenil, que notó la aparición a los 37 años de múltiples pápulas asintomáticas, duras, en ambas mejillas. El estudio histopatológico mostró que se trataba de osteomas. (AU)

Parosteal osteoma of the iliac bone.

A 33-year-old patient with a 2-year history of intermittent pain in the right gluteal region and thigh presented with a large sclerotic lesion of the iliac bone. From the findings on radiography, scintigraphy, CT and MRI, a giant parosteal osteoma was suspected. The histological examination confirmed the diagnosis. Since the lesion was extensive it was observed with periodic follow-up examinations. At present, 5 years after the diagnosis, the patient is asymptomatic and imaging studies show that the lesion persists with reduction of sclerosis and size. The tumor was on the surface as well as intramedullary--only one other case with such a distribution is known to us--and it was also in the iliac bone.

Benign skull lesions.

OBJECTIVE: To document the epidemiologic and clinical features of benign skull lesions. DESIGN: A case series. SETTING: St. Michael's Hospital, a tertiary care facility affiliated with the University of Toronto. PATIENTS: Thirty-one patients who had a neurosurgical consultation and were discharged from hospital after excision of a benign skull lesion during a 10-year period. MAIN OUTCOME MEASURES: Patient demographics, clinical signs and symptoms, radiographic and pathological tumour characteristics, surgical procedure, length of hospital stay, outcome and follow-up. RESULTS: The 31 patients (6 men, 25 women) had 32 lesions excised. The mean age of the patients was 41.9 years. Osteomas accounted for 63% of the tumours. The most frequent location was the parietal bone. Neurologic symptoms were absent in the majority of calvarial tumours. Useful diagnostic studies included plain skull radiography and computed tomography. Nuclear bone scanning was done in 7 patients. All patients underwent craniectomy, with cranioplasty in most cases. Three patients had new neurologic symptoms postoperatively and 1 patient had incomplete resolution of symptoms. CONCLUSIONS: Benign skull lesions are infrequent, but they require neurosurgical intervention. When necessary, surgical excision can serve to confirm the diagnosis, improve cosmesis and retard the progression of neurologic dysfunction. Of primary importance is the recognition of such lesions by primary care physicians and referral to the surgeon so that an appropriate treatment plan can be made.

Osteoma of the internal auditory canal. Case report and literature review.

Osteomas of the internal auditory canal are rare lesions, with only 12 reported cases in the world literature. Symptoms are those of eighth nerve compression, and include unilateral hearing loss and vestibular weakness, thus mimicking symptoms of acoustic neuroma. We report a patient with an osteoma of the internal auditory canal, along with a review of the literature. We note age and sex characteristics from the literature, give evidence of localized trauma as a possible etiologic factor for this lesion, and discuss the pitfalls of relying exclusively on magnetic resonance imaging in the workup of suspected retrocochlear lesions.

[Osteomas and exostoses of the facial structures: a morphological study and the etiopathogenetic considerations]. / Osteomi ed esostosi del massiccio facciale: studio morfologico e considerazioni etiopatogenetiche.

The differential diagnosis of the osteocondensing lesions (osteomata and exostoses) is till today a topical issue for the experts. In order to test the reliability of the etiopathogenetic theories advanced up to now, the authors carried out a research on samples of tissue coming from surgical interventions for osteomata and exostoses of the maxillofacial region resortied with optical and polarized-light microscopes (OM-PLM). This research meant to analyze the morphological and structural characteristics of newly formed tissues; a considerable attention has been devoted to the difference between primary and secondary bone and to the quality of cementing lines. Thanks to the data resulted from this study, the authors advanced interesting theories about these pathologies either from an etiological and nosological point of view.

Paranasal sinus osteomas: a review of 46 cases.

In a survey of 1500 coronal sinonasal CT scans, 46 patients were found to have paranasal sinus osteomas. The overall incidence was 3%, with a predilection for decades 5 and 6 and a male-to-female ratio of 1.3:1. The frontal sinus was most commonly involved, and of these lesions 37% were in the immediate vicinity of the nasofrontal duct and 21% above and lateral to the ostium. Tumour size varied from a mean diameter of 1.5 mm to 30 mm; however, 17 lesions were 5 mm in size or less. Five matrix patterns were observed. Only two patients were symptomatic and only three osteomas excised. Isotope bone scanning may be a useful screening test with specific follow-up utilising computed tomography.

Ossifying fibroma of the jaws: a case report.

This communication presents the first case report of ossifying fibroma of the jaw from Kenya and reviews the literature including the available published case reports from Africa. The relationship between ossifying fibroma and other related benign fibro-osseous lesion of the jaws is briefly discussed. Intensified research work on this condition in Africans is called for so that more cases should be published since it is reportedly more common in black race. The majority of the African cases are generally reported in advanced stages indicating neglect or delay in seeking for medical help. Since ossifying fibroma is a well encapsulated and expansile benign bone neoplasm, surgical enucleation appears to be the treatment of choice; recurrence is rare.