Microbiology … Del Fattore et al. Now customize the name of a clipboard to store your clips. with ADO type II and fractures in ≤3 bones was reported in 20% of the patients. Sign up here as a reviewer to help fast-track new submissions. Family history of the patient was unclear.
ADO type I is generally very mild with a diffuse sclerosis without alterations in the bone turnover. Severe visual loss was reported in 19% of the patients by Waguespack et al. discussed 62 cases with ADO type II, among these 19 patients were ≤18 years of age and 43 patients were >18 years of age [21]. The patient was prescribed Volini gel (diclofenac diethylamine 1.16%, methyl salicylate 10%, and linseed oil 3%) for topical application twice daily on the lower back. Radiographs reveal sclerosis of the skull, which mainly results in increased thickness of the cranial vault; however, the spine does not show much sclerosis.
In most patients, this pattern is inherited as an autosomal dominant trait and has been termed benign osteopetrosis. Patient gave no history of previous fractures or swellings elsewhere in the body. Erlenmeyer-shaped femoral metaphysis and transverse bands of osteosclerosis are observed in the long bones. Panoramic radiograph of the patient’s brother was also performed, which was normal (Figure 13).
However, 20%–40% of the patients remain asymptomatic [17, 18].
Other investigations, that is, renal function test, liver function test, ESR, serum calcium, and serum phosphorus, were also within the normal limit. Peripheral smear showed normocytic and normochromic anemia. Clinical manifestation in the form of diffuse pain was reported in 30% of the patients by Del Fattore et al. The patient also maintained a good oral hygiene. reported generalised osteosclerosis in 43% of the cases, rugger jersey spine was noted in 52.3% of the patients, and 33.3% patients had bone-in-bone appearance [19]. bachelor, undergraduate etc). Del Fattore et al. El-Tawil and Stoker reported a fracture rate of 62% (femur most common) [24]. This phenotype is not associated with an increased fracture rate and is reported to be fully penetrant [8]. Right side of the mandible showed obliteration of the inferior alveolar canal, and cotton wool appearance of the trabeculae was noted at the right angle of the mandible. The base of the skull appeared highly radiodense with loss of trabecular pattern, and there was hypoplasia of foramen magnum, and other foramina were obliterated (Figure 3). (A) Panoramic radiograph demonstrating mixed radiolucent and radiopaque lesions in the mandible with "cotton wool" appearance.Lesions are well demarcated with a radiolucent ring in all four quadrants though they are more subtle in the maxilla (B) Axial CT scan image showing … Autosomal dominant osteopetrosis exhibits a heterogeneous trait with milder symptoms, often at later childhood or adulthood. Many patients undergo several orthopaedic procedures that are often complicated owing to the hardness and the brittleness of their skeletons. 10% of the patients reported fractures in 4–10 bones, and more than 10 bones were fractured in 15% of the patients [19]. Early death in patients with ADO type II is rare, but some patients can experience a very poor quality of life [16].
Right side of the mandible showed obliteration of the inferior alveolar canal, and cotton wool appearance of the trabeculae was noted at the right angle of the mandible. No caries, mobility, attrition, abrasion, and so forth were noted in any of the teeth. In case you need help on any kind of academic writing visit website ⇒ www.WritePaper.info ⇐ and place your order, Isra Institute of Rehab Sciences (IIRS), Isra University, No public clipboards found for this slide. Priyanka Kant, Neelkamal Sharda, Rahul R. Bhowate, "Clinical and Radiological Findings of Autosomal Dominant Osteopetrosis Type II: A Case Report", Case Reports in Dentistry, vol. Mcq Added by: EHAB KHAN. A provisional diagnosis of chronic generalised periodontitis was made. The more time you have before the deadline - the less price of the order you will have. Dental problems like delayed tooth eruption, ankylosis, abscesses, cysts, and fistulas are common in patients with benign osteopetrosis.
The differential diagnosis that can be considered includes other sclerosing bone dysplasias, such as pycnodysostosis, craniometaphyseal dysplasia, diaphyseal dysplasia, melorheostosis, osteopoikilosis, and osteopathia striata. Benign osteopetrosis is a rare disorder, which might be characterized by an asymptomatic clinical picture. Active osteoclasts. Therefore, routine dental surveillance and maintenance of oral hygiene form an integral part of management and play an important role in preventing more severe complications such as osteomyelitis of the mandible [12]. Hematologic investigations showed Hb of 10.8 g/dL, white blood cells 6.5 k/uL, and platelet count 130 k/uL, and ESR was in the normal range.
A review of the systems was noncon- tributory. Cotton wool appearance is seen _____ ? Our case had no signs and symptoms of osteomyelitis affecting any of the bones. Visual impairment and central nervous system involvement is rare in autosomal dominant osteopetrosis type II [12]. Constriction of the inferior alveolar nerve canal and dental pulp canal and thickening of the lamina dura are routinely seen in dental radiographs because of increased bone density.
A 35-year-old female patient reported with the chief complaint of deposits on teeth from the past 6-7 months. No signs of cranial nerve involvement, dental caries, and abscesses were noted. However, 55% of the patients reported by Del Fattore et al. Radiograph of the hip joint showed generalised sclerosis of the pelvic rami and the femur bone (Figure 10). We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. Treatment required in more extensive cases must be as aggressive as dictated by the entire disease process and the total medical evaluation. Growth Disorders And Neoplasia Growth Disorders And Neoplasia Medical Mcqs Medical Mcqs for Exams preparation of Medical Students and professionals. Bénichou et al. Most individuals diagnosed with autosomal dominant osteopetrosis type II have an affected parent, although in our case the family history was unclear, and a panoramic radiograph of the patient’s brother was performed which was normal. Radiograph of the humerus showing dense sclerosis of the humerus and scapula and “funnel-like appearance” in the humerus (Figure 11). Diffuse patchy, cotton-wool appearance in sclerosis of bone.
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Cranial nerve compression is rare, hearing loss and visual loss occur in fewer than 5% of the affected patients. Moreover, because of the high infection risk and increased susceptibility to jaw fracture in these patients, they should receive increased attention and prophylactic dental treatment to maintain their fragile oral health status. Left side of the mandible was comparatively less affected, showing an increase in bone density but no cotton wool appearance and no frank obliteration of the inferior alveolar canal. 2013, Article ID 707343, 8 pages, 2013. https://doi.org/10.1155/2013/707343, 1Department of Oral Medicine and Radiology, Manav Rachna Dental College, Sector 43, Aravalli Hills, Delhi-Surajkund Road, Faridabad, Haryana 121001, India, 2Department of Oral Medicine and Radiology, Sharad Pawar Dental College and Hospital, Sawangi, Wardha, Maharashtra 442001, India, Osteopetrosis is a rare inherited genetic disease characterized by sclerosis of the skeleton caused by the absence or malfunction of osteoclasts. Autosomal dominant osteopetrosis type II was the first type of osteopetrosis recognized and described by Albers-Schonberg in 1904 [1]. Del Fattore et al. The term osteopetrosis is derived from the Greek word “osteo” meaning bone and “petros” meaning stone. There was no history of trauma and fractures in any other part of the body. Supragingival, subgingival scaling, and root planning were done for the patient. Slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. This report describes the long term management of a particularly aggressive case of CSO and indicates maxillary sinus disease as a possible etiologic factor in CSO of the maxillary alveolus. Generalised marked increase in the bone density, lack of distinct lamina dura, and altered trabecular pattern were noted in both jaws; the trabeculae were coarse, dense, and increased in number. Clipping is a handy way to collect important slides you want to go back to later. Waguespack et al. These sclerotic patches are poorly defined and fluffy.
Osteopetrotic conditions vary greatly in their presentation and severity, ranging from neonatal onset with life-threatening complications such as bone marrow failure (e.g., classic or “malignant” ARO) to the incidental finding of osteopetrosis on radiographs (e.g., osteopoikilosis) [12]. At present, no effective medical treatment for osteopetrosis exists. Formerly Senior Resident; currently in private practice in High Point, N. C. We use cookies to help provide and enhance our service and tailor content and ads. ADO type II is the most common form of osteopetrosis with an estimated prevalence of 1 in 20,000 births. If you wish to opt out, please close your SlideShare account. Our patient was a 35-year-old female.
However, the radiological penetrance is only 60%–90% [12]. Clinical manifestations of ADO type II are dominated by long-bone fractures [19]. Chronic osteomyelitis is a primary infection of the mandible presenting with unilateral pain, soft tissue swelling, fever, and lymphadenopathy. These views revealed thickening of the cortical boundaries of all the bones with obliteration of the marrow spaces. Peripheral smear showed normocytic normochromic anemia. See also. Chronic sclerosing osteomyelitis has been frequently described in the literature but little has been recommended for the management of the resistent cases that fail to respond to conservative treatment.
As of this date, Scribd will manage your SlideShare account and any content you may have on SlideShare, and Scribd's General Terms of Use and Privacy Policy will apply. Due to therarity of the disease and paucity of reported cases of autosomal dominant osteopetrosis type II in the literature, the purpose of this paper is to report a case of ADO type II in a 35-year-old female patient. Indistinct often Usually mimic the diseases ; Paget's disease, cemento-osseous dysplasia. the endosteum) of bones, most typically long bones, due to slow-growing medullary lesions..
This disease has been reported in three clinical forms: (1) malignant infantile form with poor prognosis and autosomal recessive inheritance, (2) benign/adult osteopetrosis with autosomal dominant inheritance and with fewer symptoms, (3) autosomal recessive intermediate form with clinical manifestations similar to malignant form and lowest incidence rate [8–10]. Maxillary sinuses also appear hypoplastic [26]. Renal function and serum electrolytes were within normal limits (creatinine 0.61 mg/dL, sodium 136 mEq/L, and potassium 3.9 mEq/L). Orthopanogram showing cotton-wool appearance of the maxilla and mandible as well as hypercementosis of several teeth. I'd advise you to use this service: ⇒ www.HelpWriting.net ⇐ The price of your order will depend on the deadline and type of paper (e.g. Copyright © 1980 Published by Elsevier Inc. Radiograph of the lumbar spine AP and lateral view showed sclerosis at all the levels and “bone-in-bone appearance.” Normal spinous processes, normal height, and morphology of the individual vertebrae were noted (Figures 7 and 8).
If osteomyelitis occurs, surgical intervention must be considered because antibiotics do not reach the compromised region [17]. MEDICAL MCQS; 1. 8, 10 The secondarily infected lesion of FOD has a wider radiolucent border than chronic osteomyelitis. were males and 45% of the patients were females [19].
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