FACOMATOSIS CLASIFICACION PDF

Introduction and objectives: The juvenile nasopharyngeal angiofibroma is a highly vascularised benign neoplasm of complex treatment in its surgical preparation, surgery to be performed, risks and recurrences. The aim of the study was to analyze the management and surgical treatment for the pathology of juvenile nasoangiofibroma. Materials and methods: We reviewed the clinical histories and images of the patients who underwent surgery with a pathology result of juvenile nasoangiofibroma in the period from January to December Results: Sixty-one cases were treated; all of them treated using the same surgical access by means of a Le Fort I osteotomy.

Author:Zulkijin Dulkis
Country:Kenya
Language:English (Spanish)
Genre:Photos
Published (Last):5 March 2014
Pages:358
PDF File Size:13.82 Mb
ePub File Size:5.87 Mb
ISBN:605-1-73228-426-4
Downloads:65737
Price:Free* [*Free Regsitration Required]
Uploader:Kegis



Introduction and objectives: The juvenile nasopharyngeal angiofibroma is a highly vascularised benign neoplasm of complex treatment in its surgical preparation, surgery to be performed, risks and recurrences.

The aim of the study was to analyze the management and surgical treatment for the pathology of juvenile nasoangiofibroma. Materials and methods: We reviewed the clinical histories and images of the patients who underwent surgery with a pathology result of juvenile nasoangiofibroma in the period from January to December Results: Sixty-one cases were treated; all of them treated using the same surgical access by means of a Le Fort I osteotomy. All of the patients were male, with an average age of Conclusions: The described approach provided extensive surgical access, which was adequate for the different stages of the tumour.

It requires experience to be able to resect the tumour with the least possible bleeding. Tienen gran tendencia al sangrado y su tratamiento es complejo. Resultados: Se incluyeron un total de 31 pacientes, 30 varones y una mujer. Excepto en un caso, no se observaron complicaciones significativas. El seguimiento medio fue de 86 meses. Background and objectives: Juvenile angiofibroma JA is a benign tumour, for which the treatment of choice is surgery. It may be associated with significant morbidity because of its anatomical location and its locally destructive growth pattern.

Severe haemorrhage constitutes a high risk in JA and its surgical management can be complex. The management of JA remains a challenge. Material and methods: Medical records of patients operated for JA were reviewed. Results: A total of 30 male patients and one female were included. The mean age was 17 years. The mean blood loss was 1. Mean postoperative follow-up time was 86 months. Conclusions: This retrospective study supports the notion that endonasal endoscopic approaches for a JA are a feasible option associated with good long-term results.

Their topical use is recent and poorly codified. Reports of all studies investigating the use of topical mTOR inhibitors in any dermatology diseases were included. The exclusion criteria were systemic use and mucosal administration. In all, 11 dermatologic conditions were found, the most frequent being angiofibromas linked to tuberous sclerosis complex patients.

Topical mTOR inhibitors were significantly more efficient than placebo for angiofibromas relative risk, 2. The median concentration of sirolimus was 0. Topical mTOR inhibitors were well tolerated, with only mild or moderate local side effects mostly irritative reported. Other indications require further research. Extranasopharyngeal angiofibroma ENA is an angiofibroma that occurs in the extranasopharynx. It shows pathologic findings, such as juvenile angiofibroma JNA , which accounts for 0.

However, compared with JNA, the prevalence, affected site, and clinical characteristics are completely different, which leads some physicians to classify ENA as a disease different from JNA. ENA of the nasal turbinate origin are rarely reported in the literature. In addition, choanal polyp originating from the posterior part of the nasal turbinate is uncommonly reported. Recently, we encountered two cases of ENA, which were not diagnosed by intraoperative frozen section examination, but were histopathologically diagnosed post-operatively.

Although we were unable to diagnose and perform embolization pre-operatively, ENA has been successfully treated by endoscopic surgery, without profuse bleeding. Nasal obstruction is one of the most common problems seen by pediatric otolaryngologists. Prompt treatment of nasal obstruction can be critical in newborns and infants because of their obligatory nasal breathing.

Older children will typically have more inflammatory, infectious, or traumatic causes of nasal obstruction. Nasal obstruction can lead to a significant decrease in the quality of life in children along with an increase health care expenditures. METHODS: This clinical record elucidates the only case of extra-nasopharyngeal angiofibroma arising from the septum in a female child, who presented with epistaxis.

RESULTS: The histopathological diagnosis was confirmed by immunohistochemistry, and the case was managed surgically with no recurrence. The aim of this study was to analyze the clinical and genetic features of TSC and to assess the treatment of facial angiofibromas using topical sirolimus in Chinese children.

Genetic analyses were performed in 12 children and their parents. Children were treated with 0. There was no detectable systemic absorption of sirolimus. Genetic testing has a high detection rate in patients with a clinical diagnosis of TSC. Topical sirolimus appears to be both effective and well-tolerated as a treatment of facial angiofibromas in children with TSC. The response typically plateaus after 12 to 24 weeks of treatment.

All patients received radiologic examinations and surgical excision for histopathology and immunohistochemistry examinations. Five patients had no recurrence after a minimum follow-up of 12 months. One patient case 6 experienced recurrence twice, and had debulking surgeries each time.

At present, the patient still has remnant tumour in the orbit, but no growth has been detected during the past two years. The tumour size will be closely monitored.

Complete surgical excision is important for long-term prognosis, and immunohistochemical study is helpful for confirming pathologic diagnosis. Importance: Inhibitors of mammalian target of rapamycin complex 1, such as sirolimus, effectively target skin lesions in tuberous sclerosis complex TSC. However, systemic treatment causes adverse effects, and topical sirolimus has shown promise in the treatment of facial angiofibromas. Objective: To evaluate the efficacy, safety, and optimal concentration of a topical sirolimus gel vs placebo for treatment of facial angiofibromas in TSC.

Design, Setting, and Participants: A double-blind, placebo-controlled, parallel-group, dose-escalation, phase 2 randomized clinical trial using 3 sirolimus gel concentrations was performed at Osaka University Hospital, Osaka, Japan.

Thirty-six patients with TSC and facial angiofibromas, including 18 aged 3 to 18 years children and 18 aged 19 to 65 years adults , were enrolled from December 10, , to July 17, Analysis was by intention to treat. The medication was applied to the patient's lesions twice per day for 12 weeks.

Each patient underwent assessment at 2, 4, 8, and 12 weeks during treatment and at 4 weeks after discontinuation of the treatment 16 weeks. Main Outcomes and Measures: The primary end point, planned before starting data collection, was an improvement factor, represented as a variable composed of tumor size reduction and a lessening of the redness of the 3 target tumors at 12 weeks relative to baseline.

Results: All 36 patients 13 male and 23 female; median age, 40 years; range, years completed the study analyses. The improvement factor was statistically significant in all active treatment groups receiving 0. Most cases can be managed by simple anterior nasal packing. This is usually a safe and very effective option in an emergency situation, requiring minimal expertise and infrastructure.

This paper describes a rare instance of a serious complication following anterior nasal packing in a case of nasopharyngeal angiofibroma. The patient was stabilised and anterior nasal packing was performed, which controlled the bleeding. Three hours later, the patient developed complete blindness in both eyes. Aggressive medical management was initiated immediately, but failed to restore the patient's vision.

However, it can occasionally lead to serious complications such as blindness. Thus, obtaining informed consent is essential to avoid medico-legal consequences in high-risk cases. Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. MTOR activation signal has been recently described in systemic lupus erythematosus SLE and its inhibition could be beneficial in patients with lupus nephritis.

We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex TSC 30 years after the onset of SLE with severe renal disease tipe IV nephritis who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest AU. A 9 year old male presented with nasal obstruction and recurrent, unprovoked epistaxis for 1 week.

Imaging revealed a highly vascular mass in the nasopharynx. The feeding vessels were subsequently embolized and the mass was removed completely. Juvenile nasopharyngeal angiofibroma is a benign but locally invasive tumor accounting for about 0. Patients usually present with nasal obstruction and epistaxis.

The tumor can however be extensive on presentation with intra orbital and intra cranial extension. The treatment is surgical removal of the tumor and the approach depends on the size of the tumor by either endoscopic or open approach. The patients were classified according to age, sex, presenting symptom, tumor stage, surgical procedure applied, preoperative embolization, perioperative blood transfusion, complications, and the presence of recurrence.

Transnasal endoscopic approach in 31 patients, midfacial degloving in six patients, and lateral rhinotomy approach in three patients were applied. Preoperative embolization reduces the amount of perioperative bleeding. Endoscopic transnasal approach decreases the rate of complications and length of hospitalization. Study of two patients.

Acta otorrinolaringol. Ear Nose Throat J ; 98 7 : , Aug. Topical use of mammalian target of rapamycin inhibitors in dermatology: A systematic review with meta-analysis.

J Am Acad Dermatol ; 80 3 : , Mar. Acta Otorrinolaringol Esp ; 70 5 : , Acta Otorrinolaringol Esp ; 70 3 : , Extranasopharyngeal angiofibroma mimicking choanal polyp in patients with chronic paranasal sinusitis. Auris Nasus Larynx ; 46 2 : , Apr.

ELITEGROUP L7MM2 1.1 PDF

Diccionario de Medicina

Fragmen Alkitab Wulfila yang terlestarikan terdiri dari sejumlah kodeks dari abad ke-6 sampai ke-8 "Textkritische Anmerkungen zur gotischen Bibel" PDF. Peta kerja sama Prancis, aksi budaya, dan jaringan ekonomi di Indonesia. We adopt the sign conventions of Birrell and Davies [2], which are the conventions in the notation of Misner, Thorne, and Wheeler [3]. Electric actuators. Electric actuators are equipped with an electric motorgearbox combination, particularly more, certain valves are operated via lever arrangements.

INDESCRIBABLE SHANTEL TESSIER PDF

Kanski. OftalmologĂ­a clĂ­nica

Omar P. From he completed a residency in pathology at Oregon Health Sciences University in Portland, Oregon, followed by a year as the Holtzman Fellow in Dermatopathology working with the late A. At Wake, Omar is in charge of the Fellowship program and has trained more than 70 national and international fellows in Dermatopathology. In addition he has instituted an international fellowship, training both dermatologists and pathologists from all over Latin America, Spain and the Middle East in the field of Dermatopathology. In he was awarded the Walter R.

Related Articles