scholarly journals Prevention of pathological fracture in fibrous dysplasia of proximal femur in a 24-year-old female a rare presentation - a case report

Author(s):  
Amit Supe ◽  
Jayesh Mhatre ◽  
Nihar Modi

<p class="abstract">Fibrous dysplasia (FD) was historically mentioned in the medical literature in 1938 by Dr. Lichtenstein and in 1942 by Dr. Lichtenstein and Jaffe. The term Jaffe-Lichtenstein syndrome is sometimes used synonymously with monostotic FD or to denote cases of polyostotic FD with café au lait spots, but no endocrine dysfunction. Our case is a 24-year-old female, housewife presented to our hospital with a complaints of right hip pain and backache with difficulty in walking of 6 months duration. She had dull aching pain which increased in intensity with the course of time, there was no radiation, but was aggravated by movements. She was managed with dynamic hip screw along with curettage of the lesion and bone graft, after 9 months of follow up, she had no pain over her hip and there was calcification over the previous lesion, our patient had a very favourable outcome.</p>

2021 ◽  
Author(s):  
Alhad Mulkalwar

UNSTRUCTURED A case report is a detailed narrative that describes the symptoms, signs, diagnosis, treatment, and follow-up of a medical problem of an individual patient. They usually describe an unusual or novel occurrence and although they lie at the bottom of the hierarchy of the Evidence Based Medicine Pyramid, they still remain one of the cornerstones of medical progress and provide key additions to the existing medical literature. Unfortunately, abysmally few physicians-in-training receive a formal education regarding what constitutes a publishable case report. The article throws light on this aspect of medical education


2016 ◽  
Vol 88 (4) ◽  
pp. 330 ◽  
Author(s):  
Luca Leone ◽  
Paola Fulvi ◽  
Giulia Sbrollini ◽  
Alessandra Filosa ◽  
Enrico Caraceni ◽  
...  

Introduction: Testicular benign tumors are very rare (&lt; 5%). Testicular Angiofibroma (AF) is one of those, however the gold standard of treatment and follow-up is still unclear. Case report: A 47 years-old man with only one functioning testis was referred to our clinic for a palpable right testicular mass and atrophic contralateral testis. Patient underwent testis-sparing surgery with inguinal approach and intraoperative frozen sections examination with diagnosis of AF. Final histology confirmed AF. Post-operative follow-up was uneventful. Clinical and ultrasonographic follow-up was negative after 8 months. Conclusion: We report a conservative surgery in a patient with AF of the solitary testis. AF is a benign para-testicular fibrous neoplasm that could be misinterpreted as malignant tumor and treated with orchiectomy. Testis-sparing surgery is recommended in this case with intraoperative pathological examination. The excision of the mass is enough but in front of a possible recurrence a long follow-up is advisable.


2020 ◽  
Vol 40 (2) ◽  
pp. 134-137
Author(s):  
Subhana Thapa Karki ◽  
Vandana Jain

McCune Albright syndrome (MAS) is a very rare genetic disease characterised by any two of the following three findings: café au lait spots, polyosteotic fibrous dysplasia and endocrine disorders. The clinical presentation of MAS may vary depending on which of the various components of the syndrome predominate. Here, we report one case of MAS presenting with precocious puberty.


2019 ◽  
Vol 16 (1) ◽  
pp. 70
Author(s):  
MoshoodFolorunsho Adeyemi ◽  
MoninuolaAdebusola Ernest ◽  
Ehigie Igben ◽  
HabibAyodeji Adeyemi

2020 ◽  
Vol 8 ◽  
Author(s):  
Sujit Kumar Tripathy ◽  
Shakti Swaroop ◽  
Sandeep Velagada ◽  
Debashree Priyadarshini ◽  
Rashmi Ranjan Das ◽  
...  

Objective: This retrospective study evaluated the outcome and safety of long-term treatment with zoledronic acid, in both polyostotic and mono-ostotic fibrous dysplasia (FD) of children.Methods: The case records of children and adolescents with symptomatic FD who received zoledronic acid (0.1 mg/kg IV infusion over 1 h) and have completed at least 2 years follow-up were analyzed. The relevant details were recorded in a predesigned chart. Clinical assessment [pain assessment by visual analog scale (VAS) and incidence of new fracture], radiological changes (cortical thickening, ossification, and decrease in the diameter of the osteolytic lesions) and biochemical parameters [alkaline phosphatase (ALP)] were used to evaluate the improvement.Results: The mean age of presentation was 9.1 years, with four males and six females. All patients had symptomatic FD in the lower limb with complaints of pain, tenderness, swelling, or deformity. Four children had associated pathological fracture. The radiological evaluation with bone scan revealed polyostotic involvement in eight patients and mono-ostotic involvement in two patients. Three patients had associated systemic features like café-au-lait spots or precocious puberty. The fracture united within 3 months and the radiological improvement was evident in the form of filling of the osteolytic defect. The pain score in six patients showed significant improvement (VAS &lt; 3). The ALP level decreased to 544.12 ± 47.35 IU/L from an initial value of 895.75 ± 79.64 IU/L (p = 0.04) at 12 months. One patient had symptomatic hypocalcaemia after zoledronic acid infusion.Conclusion: The clinical and radiological response of zoledronic acid treatment in FD of children is promising. Further randomized control trials with a larger sample size are required to establish this drug as a first-line medical treatment in FD.


Author(s):  
Pratyush Shahi ◽  
Apoorv Sehgal ◽  
Aarushi Sudan

A 35-year-old female presented to us with anterior bowing of her right tibia. The deformity developed in her adolescence and subsequently had not progressed for nearly two decades. The patient had no functional limitation, her only concern being cosmesis. Radiological investigations suggested either fibrous dysplasia or adamantinoma. Biopsy showed fibrous stroma consisting of myxofibrous tissue and woven bone which was confirmatory of fibrous dysplasia. Keeping in mind that it was a dormant benign lesion not hindering with functionality of the limb, it was decided to keep the patient under observation with regular follow-up.


Author(s):  
Preeti B. Singh ◽  
Roli Purwar ◽  
Pawan Kumar

Gonadal dysgenesis is a group of heterogeneous disorders with very rare presentation. The spectrum of disease not only includes primary amenorrhoea but also secondary amenorrhoea. Herein, we are reporting a case of 16-year-old phenotypic female who presented with amenorrhoea with 46, XX karyotype with hypoplastic uterus with absent ovaries (on imaging), with high gonadotropins level and low estradiol. Suspecting Mayer–Rokitansky–Küster–Hauser syndrome (due to hypoplastic uterus) with gonadal dysgenesis she was started on cyclic hormones for development of secondary sexual characters and to prevent bone loss. But, during follow up, after giving estrogen for 8 months, her hypoplastic uterus again starts reappearing with attainment of cyclic menses on estrogen and progesterone withdrawal. We concluded that, in the presence of rudimentary or hypoplastic uterus, straightforward diagnosis of MRKH is to be avoided without seeing peripheral estrogenisation, hormone profile and karyotype analysis.  


2020 ◽  
pp. 1-3
Author(s):  
M. Darrsana ◽  
Venkatachalam. K ◽  
Ampalaya Manu R ◽  
Haemanath P

Fibrous dysplasia is an ailment , that can include , one or a few bones and is portrayed by bone deformations, pain and fractures, arising due to bone weakening. A few patients can also present with endocrine dysfunction ( precocious puberty) and cutaneous cafe '- au - lait spots.[1] Determination depends on X-ray assessment. Anticipation is to be surveyed with X-rays and markers of bone remodelling. A few newer comprehensions , of the patho - physiology have been made in the past I0 years. It is presently perceived that fibrous dysplasia is brought about , by a physical initiating transformation of the Gs alpha subunits , of the protein G, bringing about an expanded cAMP fixation and in this way, brings about anomalies of osteoblasts separation, and then subsequently these osteoblasts, creates abnormal bone. There is also an expansion in interleukin-6-initiated osteoclastic bone resorption, which forms the basis of reasoning, for treating these patients with bisphosphonates. In the previous 10 years, the bisphosphonate pamidronate has been utilized by imbuement for fibrous dysplasia (two courses for each year), with great outcomes regarding pain and, in about half of patients, the topping off of osteolytic sores.[2]


2012 ◽  
Vol 126 (8) ◽  
pp. 830-832
Author(s):  
J P Jacobson ◽  
A Weisstuch ◽  
C Hajdu ◽  
D Myssiorek

AbstractObjective:To report a case of sebaceoma of the auricle, and to discuss the differential diagnosis, histopathological features, surgical management and genetic associations of this entity.Methods:Case report and review of the medical literature.Results:A 79-year-old man presented with a slowly growing lesion of his auricle. Excision of the mass and histopathological review revealed a benign, basaloid, adnexal neoplasm consistent with sebaceoma. Due to its association with Muir–Torre syndrome and increased risk of visceral malignancy, the patient was followed closely for signs of malignancy. At 36 months post-excision, there were no signs of recurrence; thereafter, the patient continued to receive routine cancer surveillance follow up.Conclusion:Sebaceoma is a rarely encountered, benign, adnexal neoplasm which can occur in the head and neck. The treatment is surgical excision, and recurrence is rare. Sebaceoma can occur as part of Muir–Torre syndrome, and in these patients there is an increased risk of other sebaceous lesions and visceral malignancy; thus, genetic testing and surveillance should be strongly considered.


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