Orbital lymphomatoid granulomatosis - a rare cause of proptosis

Energy Technology Data Exchange (ETDEWEB)

Du Toit, Jacqueline; Kilborn, Tracy [Department of Radiology, Red Cross Children' s Hospital, Rondebosch (South Africa); Eyssen, Ann van [Department of Oncology, Red Cross Children' s Hospital, Rondebosch (South Africa); Pillay, Komala [Department of Pathology, Red Cross Children' s Hospital, Rondebosch (South Africa)

2015-07-15

A 1-year-old girl with unilateral proptosis was found to have primary orbital lymphomatoid granulomatosis - a condition rarely occurring in children. This multisystem angiocentric, angiodestructive, lymphoproliferative disease typically involves the lungs, with ocular involvement being extremely uncommon. Our case serves to illustrate the imaging findings of this unusual condition and highlight a rare cause of proptosis. (orig.)

Two rare cases of laryngeal intralymphatic histiocytosis

DEFF Research Database (Denmark)

Reznitsky, Martin; Daugaard, Søren; Charabi, Birgitte Wittenborg

2016-01-01

We report two rare cases of intralymphatic histiocytosis causing, respectively, recurrent and persistent episodes of upper airway swelling and breathing difficulties. Case 1 was a 39-year-old man who was referred with recurrent upper airway swelling causing difficulty in breathing. A direct....... Extensive investigations were performed but discovered no abnormal findings. He received CO2 laser treatment twice and the swelling decreased. Intralymphatic histiocytosis is extremely rare in upper airway pathology. It is an important differential diagnosis in patients with recurrent and chronic laryngeal...

Scapulothoracic Dissociation: A Rare Variant: A Case Report

Directory of Open Access Journals (Sweden)

Rajat Jangir

2014-07-01

Full Text Available Scapulothoracic dissociation is a rare injury involving separation of scapula from the thorax along with the upper extremity. Majority of the patients have concomitant neurovascular injury and the prognosis is uniformly poor in such cases. We present a case of scapulothoracic dissociation with comminuted fracture of scapula and acromioclavicular joint disruption without neurovascular deficit. There were associated avulsion fractures of the spinous processes of vertebrae (T3-T5. Such presentation is rare in an already rare scapulothoracic dissociation injury. A discussion regarding the probable mechanism of injury, management and prognosis is presented.

Epistaxis as a Common Presenting Symptom of Glanzmann’s Thrombasthenia, a Rare Qualitative Platelet Disorder: Illustrative Case Examples

Directory of Open Access Journals (Sweden)

Michael Recht

2017-01-01

Full Text Available Children often present to emergency departments (EDs with uncontrollable nose bleeding. Although usually due to benign etiologies, epistaxis may be the presenting symptom of an inherited bleeding disorder. Whereas most bleeding disorders are detected through standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. Here we present two case reports and review diagnostic and management challenges of platelet function disorders with a focus on Glanzmann’s thrombasthenia (GT. Patient 1 was a 4-year-old boy with uncontrolled epistaxis. His medical history included frequent and easy bruising. Previous laboratory evaluation revealed only mild microcytic anemia. An otolaryngologist stopped the bleeding, and referral to a pediatric hematologist led to the definitive diagnosis of GT. Patient 2 was a 2.5-year-old girl with severe epistaxis and a history of milder recurrent epistaxis. She had a bruise on her abdomen with a palpable hematoma and many scattered petechiae. Previous assessments revealed no demonstrable hemostatic anomalies. Platelet aggregation studies were performed following referral to a pediatric hematologist, leading to the diagnosis of GT. As evidenced by these cases, the ED physician may often be the first to evaluate severe or recurrent epistaxis and should recognize indications for coagulation testing and hematology consultation/referral for advanced hematologic assessments.

Cronkhite-Canada Syndrome (CCS)-A Rare Case Report.

Science.gov (United States)

Chakrabarti, Subrata

2015-03-01

Cronkhite-Canada syndrome (CCS) is an extremely rare non-inherited condition characterized by gastrointestinal hamartomatous polyposis, alopecia, onychodystrophy, hyperpigmentation, weight loss and diarrhoea. The aetiology is probably autoimmune and diagnosis is based on history, physical examination, endoscopic findings of gastrointestinal polyposis, and histology. The disease is very rare; approximately 450 cases of CCS have been reported worldwide. The author reports a case of CCS in an elderly Indian male.

Comparison between Three Rare Cases of Co‑Infection with ...

African Journals Online (AJOL)

Co‑infection in immunocompetent patients is rare. Though co‑infection with dengue and leptospira cases is increasingly reported, a co‑infection of this combination along with hepatitis E is rarely thought of. Until date only two case of triple co‑infection have been reported world‑wide. Here, we are reporting a patient with ...

Internal hernia due to adjustable gastric band tubing: review of the literature and illustrative case video.

Science.gov (United States)

Hamed, Osama H; Simpson, Lashondria; Lomenzo, Emanuele; Kligman, Mark D

2013-11-01

Laparoscopic adjustable gastric banding (LAGB) is a commonly performed bariatric procedure. Device-related morbidity is typically associated with the subcutaneous port or the band itself. Complications related to band tubing are unusual. Small bowel obstruction (SBO) after LAGB is a unique and serious complication; there is the potential of delayed diagnosis and the risk of closed-loop bowel obstruction. SBO secondary to internal hernia caused by band tubing is very rare, with only five cases reported in the literature. In this article, we describe our experience and provide an illustrative video of a case of SBO related to band tubing. We also provide a detailed review of the few previously published case reports. Based on the common features of our case and other published case reports, we hypothesize some risk factors that might lead to this unique morbidity of adjustable gastric band tubing and provide potential solutions to prevent this problem. Tubing-related SBO is a serious complication with the risk of closed-loop bowel obstruction. Urgent operative exploration is required to avoid bowel strangulation. To prevent recurrence we advise functionally shortening the tubing by tucking it to the right upper quadrant above the liver and also provide some omental coverage between the bowel and band tubing if possible.

A rare case of acute lymphoblastic leukaemia with hemophilia A

Directory of Open Access Journals (Sweden)

John Biju

2009-12-01

Full Text Available Abstract A rare case of Acute lymphoblastic leukemia with hemophillia in a 12 year old boy is presented in the article. Patient was known case of hemophillia (factor VIII deficiency. He was diagnosed as a case of ALL based on bone marrow examination and immunophenotypic study. Patient was treated as per Children Cancer group guidelines. The main aim of reporting this rare association lies in developing treatment strategies in preventing life threatening bleeding due to this rare association which though may be accidental but need further research.

Atlas of Illustrative Cases of Tattoo Complications

DEFF Research Database (Denmark)

Serup, Jørgen

2017-01-01

proposed Bispebjerg University Hospital ('Tattoo Clinic') diagnostic system of tattoo complications, a system reported to the 11th revision of the WHO disease classification system. Cases include allergies predominantly seen in red tattoos manifesting the characteristic patterns of plaque elevation......'. Bacterial infections are also illustrated. The technical hazards of tattooing and sequels as well as unwanted effects of tattoo removal by lasers are shown along with severe scarring following tattoo removal by caustics. This atlas is a tutorial in modern medical and surgical treatments of tattoo...

Contemporary Psychoanalytic Perspectives on Eating Disorders: A Case Illustration.

Science.gov (United States)

Lerner, Howard D.

This paper examines recent formulations derived from different lines of conceptual development within psychoanalytic theory in relation to the anorexic and bulimic syndromes. The case history, clinical picture, and course of treatment of a bulimic adolescent girl are reviewed. This discussion illustrates the profound consequences upon cognition of…

Nevoid basal cell carcinoma syndrome with medulloblastoma in an African-American boy: A rare case illustrating gene-environment interaction

Energy Technology Data Exchange (ETDEWEB)

Korczak, J.F.; Goldstein, A.M. [National Institutes of Health, Bethesda, MD (United States); Kase, R.G. [Westat Inc., Rockville, MD (United States)] [and others

1997-03-31

We present an 8-year-old African-American boy with medulloblastoma and nevoid basal cell carcinoma syndrome (NBCCS) who exhibited the radiosensitive response of basal cell carcinoma (BCC) formation in the area irradiated for medulloblastoma. Such a response is well-documented in Caucasian NBCCS patients with medulloblastoma. The propositus was diagnosed with medulloblastoma at the age of 2 years and underwent surgery, chemotherapy, and craniospinal irradiation. At the age of 6 years, he was diagnosed with NBCCS following his presentation with a large odontogenic keratocyst of the mandible, pits of the palms and soles and numerous BCCs in the area of the back and neck that had been irradiated previously for medulloblastoma. Examination of other relatives showed that the propositus mother also had NBCCS but was more mildly affected; in particular, she had no BCCs. This case illustrates complex gene-environment interaction, in that increased skin pigmentation in African-Americans is presumably protective against ultraviolet, but not ionizing, radiation. This case and other similar cases in the literature show the importance of considering NBCCS in the differential diagnosis of any patient who presents with a medulloblastoma, especially before the age of 5 years, and of examining other close relatives for signs of NBCCS to determine the patient`s at-risk status. Finally, for individuals who are radiosensitive, protocols that utilize chemotherapy in lieu of radiotherapy should be considered. 27 refs., 4 figs.

Inclusion and exclusion in the globalisation of genomics; the case of rare genetic disease in Brazil.

Science.gov (United States)

Gibbon, Sahra; Aureliano, Waleska

2018-04-01

Within the context of a globalising agenda for genetic research where 'global health' is increasingly seen as necessarily informed by and having to account for genomics, the focus on rare genetic diseases is becoming prominent. Drawing from ethnographic research carried out separately by both authors in Brazil, this paper examines how an emerging focus on two different arenas of rare genetic disease, cancer genetics and a class of degenerative neurological diseases known as Ataxias, is subject to and a product of the dynamics of inclusion and exclusion as this concerns participation in research and access to health care. It examines how in these different cases 'rarenesss' has been diversely situated and differently politicised and how clinicians, patients and their families grapple with the slippery boundaries between research, rights to health and the limits of care, therapy or prevention. It illustrates how attention to rare genetic disease in Brazil emerges at the intersection of a particular history of genetic research and public health infrastructure, densely complicated feedback loops between clinical care and research, patient mobilisation around the 'judicialisation' of health and recent state legislation regarding rare disease in Brazil. It highlights the relevance of local configurations in the way rare genetic disease is being made relevant for and by different communities.

A Rare Cause of Abdominal Pain in Childhood: Cardiac Angiosarcoma

Directory of Open Access Journals (Sweden)

Elvan Caglar Citak

Full Text Available Abstract Cardiac angiosarcomas are extremely rare in childhood, they are rapidly progressive tumours that often present themselves as diagnostic dilemmas, resulting in delayed diagnosis. Also, extracardiac manifestations, including abdominal pain, are extremely rare in patients with intracardiac tumors. We herein present the case of a 15-year-old girl who presented with abdominal pain. Echocardiography and thoracic computed tomography showed right atrial mass. The patient underwent surgery, chemotherapy, and radiotherapy. Eight months after treatment, abdominal recurrence was detected. The abdominal mass was resected, and radiotherapy and new chemotherapy protocol were given. The present case illustrates a rare case of primary cardiac angiosarcoma posing a diagnostic dilemma in an adolescent girl.

'Case reporting of rare adverse events in otolaryngology': can we defend the case report?

LENUS (Irish Health Repository)

Dias, Andrew

2012-01-31

The study of errors in medicine has proliferated since the publication of The Institute of Medicine Report \\'TO ERR IS HUMAN\\' in 2000. Case nuances and process of care issues are valuable areas to explore if the goal is to provide the health care worker with the knowledge to avoid future errors. Meta-analysis and randomized controlled trials provide a large data base of evidence towards improvement and opportunities, but it is suggested that case reports can still provide valuable clinical information. The aim is to use the published literature to produce a series of rare harm case reports in E.N.T. The methods include systematic literature review. Journals searched in PUBMED were 60. Rare harm case obtained from the search were 5,322. Rare harm case reports not reported in any other form of evidence-based medicine were 40. Yes, the case report can be defended as it is an important pillar of evidence-based medicine.

Glomus tumor of penis- A rare case

Directory of Open Access Journals (Sweden)

Madhuri S. Kate

2013-01-01

Full Text Available Glomus tumors are rare, painful, and benign soft tissue tumors of the skin arising from the glomus body, an arteriovenous thermoregulatory structure. This lesion is usually found on the extremities. Glomus tumor involving the external genital organs including penis is extremely rare. Until now, only few cases have been reported in the available literature. A 22 year old male patient presented with a painful nodule over the penis. The pathologic diagnosis was glomus tumor of the glans penis. We report herewith a case of glomus tumor of penis diagnosed on histopathologic examination. Glomus tumor, being a benign neoplasm, complete extirpation of the glomus tumor is the treatment of choice.

Spectroscopic criteria for identification of nuclear tetrahedral and octahedral symmetries: Illustration on a rare earth nucleus

Science.gov (United States)

Dudek, J.; Curien, D.; Dedes, I.; Mazurek, K.; Tagami, S.; Shimizu, Y. R.; Bhattacharjee, T.

2018-02-01

We formulate criteria for identification of the nuclear tetrahedral and octahedral symmetries and illustrate for the first time their possible realization in a rare earth nucleus 152Sm. We use realistic nuclear mean-field theory calculations with the phenomenological macroscopic-microscopic method, the Gogny-Hartree-Fock-Bogoliubov approach, and general point-group theory considerations to guide the experimental identification method as illustrated on published experimental data. Following group theory the examined symmetries imply the existence of exotic rotational bands on whose properties the spectroscopic identification criteria are based. These bands may contain simultaneously states of even and odd spins, of both parities and parity doublets at well-defined spins. In the exact-symmetry limit those bands involve no E 2 transitions. We show that coexistence of tetrahedral and octahedral deformations is essential when calculating the corresponding energy minima and surrounding barriers, and that it has a characteristic impact on the rotational bands. The symmetries in question imply the existence of long-lived shape isomers and, possibly, new waiting point nuclei—impacting the nucleosynthesis processes in astrophysics—and an existence of 16-fold degenerate particle-hole excitations. Specifically designed experiments which aim at strengthening the identification arguments are briefly discussed.

Florid Cementoosseous Dysplasia: A Rare Case Report

Directory of Open Access Journals (Sweden)

Mehmet Fatih Şentürk

2013-01-01

Full Text Available Florid cementoosseous dysplasia (FCOD is a rare, benign, fibroosseous, and multifocal dysplastic lesion of the jaw that consists of cellular fibrous connective tissue with bone and cementum-like tissue. FCOD is most commonly found in middle-aged black women, is generally asymptomatic, and is usually detected during radiological examination. FCOD associated with multiple impacted teeth and bone expansion is a very rare phenomenon, and there are only a few familial cases reported in the literature. In this report, a 35-year-old male Turkish patient is presented who was diagnosed with nonfamilial FCOD from clinical, radiological, and histopathological findings. To our knowledge this is the first case of the nonfamilial FCOD with this many impacted teeth and severely expanded bones.

Adenoid cystic carcinoma of child: a rare case.

Science.gov (United States)

Mathai, Meera; Sherubin, J Eugenia; Agnihotri, Pg; Sangeetha, Gs

2014-01-01

Adenoid cystic carcinoma (ACC) is the second most common malignant tumor affecting both major and minor salivary glands. Clinically, it is a slowly growing tumor with high propensity for local invasion, recurrence and distant metastasis. It is predominantly seen in the ffith and sixth decades of life. Here, we report a rare case of ACC affecting the right maxilla of a 12-year-old girl. How to cite this article: Mathai M, Sherubin JE, Agnihotri PG, Sangeetha GS. Adenoid Cystic Carcinoma of Child: A Rare Case. Int J Clin Pediatr Dent 2014;7(3):206-208.

A rare case of acquired lymphangioma circumscriptum of the penis.

Science.gov (United States)

Adikari, S; Philippidou, M; Samuel, M

2017-02-01

Acquired lymphangioma circumscriptum is a rare occurrence on the penis. We report a case of a 47-year-old man who presented with a single lesion of acquired lymphangioma circumscriptum on the penis resembling genital warts. We report the case to increase awareness of this rare condition which may mimic sexually transmitted infections such as genital warts.

Severe Rhabdomyolysis without Systemic Involvement: A Rare Case of Idiopathic Eosinophilic Polymyositis

Directory of Open Access Journals (Sweden)

Ayesha Farooq

2015-01-01

Full Text Available Introduction. Eosinophilic polymyositis (EPM is a rare cause of rhabdomyolysis characterized by eosinophilic infiltrates in the muscle. We describe the case of a young patient with eosinophilic polymyositis causing isolated severe rhabdomyolysis without systemic involvement. Case Presentation. A 22-year-old Haitian female with no past medical history presented with progressive generalized muscle aches without precipitating factors. Examination of the extremities revealed diffuse muscle tenderness. Laboratory findings demonstrated peripheral eosinophilia and high creatinine phosphokinase (CPK and transaminase levels. Workup for the common causes of rhabdomyolysis were negative. Her CPK continued to rise to greater than 100,000 units/L so a muscle biopsy was performed which showed widespread eosinophilic infiltrate consistent with eosinophilic polymyositis. She was started on high dose systemic corticosteroids with improvement of her symptoms, eosinophilia, and CPK level. Discussion. This case illustrates a systematic workup of rhabdomyolysis in the presence of peripheral eosinophilia. Many differential diagnoses must be considered before establishing a diagnosis of idiopathic eosinophilic polymyositis. To our knowledge, our case of eosinophilic polymyositis is unique as it presented with severe rhabdomyolysis without another organ involvement. Clinicians should maintain a high index of suspicion for this physically debilitating disease to aid in prompt diagnosis.

Inclusion and exclusion in the globalisation of genomics; the case of rare genetic disease in Brazil

Science.gov (United States)

Gibbon, Sahra

2018-01-01

ABSTRACT Within the context of a globalising agenda for genetic research where ‘global health’ is increasingly seen as necessarily informed by and having to account for genomics, the focus on rare genetic diseases is becoming prominent. Drawing from ethnographic research carried out separately by both authors in Brazil, this paper examines how an emerging focus on two different arenas of rare genetic disease, cancer genetics and a class of degenerative neurological diseases known as Ataxias, is subject to and a product of the dynamics of inclusion and exclusion as this concerns participation in research and access to health care. It examines how in these different cases ‘rarenesss’ has been diversely situated and differently politicised and how clinicians, patients and their families grapple with the slippery boundaries between research, rights to health and the limits of care, therapy or prevention. It illustrates how attention to rare genetic disease in Brazil emerges at the intersection of a particular history of genetic research and public health infrastructure, densely complicated feedback loops between clinical care and research, patient mobilisation around the ‘judicialisation’ of health and recent state legislation regarding rare disease in Brazil. It highlights the relevance of local configurations in the way rare genetic disease is being made relevant for and by different communities. PMID:29533091

Waardenburg syndrome: A rare case

Directory of Open Access Journals (Sweden)

Shivlal M Rawlani

2018-01-01

Full Text Available Waardenburg Syndrome is a rare disorder of neural crest cell development. It is genetically inherited. Varying in prevalence from 1:42000 to 1:50,000, it compromises approximately 2-5% of congenital deaf children. The syndrome is not expressed in its complete form, in about 20% cases, which adds for its heterogenisity . Even among people affected in the same family,the features do vary. Unilateral heterochromia that manifests as lighter pigmentation of one iris is associated with Waardenburg syndrome and Parry-Romberg syndrome and less commonly with Hirschsprung disease. A case of ten yrs. old boy with a typical facial profile and hearing loss is reported.

Microfibrillar cardiomyopathy: A rare case

Directory of Open Access Journals (Sweden)

Narender Kumar

2011-01-01

Full Text Available Microfibrillar cardiomyopathy is a very rare cause of restrictive cardiomyopathy (RCM. The index case was a male patient who presented with shortness of breath and pedal edema. Further clinical investigations favored a clinical diagnosis of RCM. An endomyocardial biopsy revealed subendocardial and interstitial hyaline eosinophillic material resembling amyloid that did not stain with Congo red. An electron microscopic examination showed that this material was composed of twisted linear and bundles of tangled microfibrils. The etiology of the microfibrillar deposition is currently unknown. The pathologists should entertain the diagnosis of microfibrillar cardiomyopathy in suspected cases of amyloidosis that are negative for Congo red.

A rare case of Amyand’s hernia presenting as an enterocutaneous fistula

Directory of Open Access Journals (Sweden)

L Flood

2010-09-01

Full Text Available The finding of the vermiform appendix in an inguinal hernia has an incidence of approximately 1%. The condition is given the eponymous name Amyand’s hernia. However in just 0.08% the condition is complicated by an acute appendicitis. The clinical presentation varies, depending on the extent of inflammation of the appendix and is most often misdiagnosed as an incarcerated inguinal hernia. As such it is rarely recognised prior to surgical exploration. We report a case of Amyand’s hernia in an 85 year old woman, which presented as a right groin enterocutaneous fistula. CT scanning illustrated a fistulous tract in the right groin, which communicated with the caecum and the peritoneal cavity. She underwent laparotomy, which revealed that the appendix appeared inflamed, lay in the inguinal canal and was the origin of the enterocutaneous communication.

Facial nerve hemangioma: a rare case involving the vertical segment.

Science.gov (United States)

Ahmadi, Neda; Newkirk, Kenneth; Kim, H Jeffrey

2013-02-01

This case report and literature review reports on a rare case of facial nerve hemangioma (FNH) involving the vertical facial nerve (FN) segment, and discusses the clinical presentation, imaging, pathogenesis, and management of these rare lesions. A 53-year-old male presented with a 10-year history of right hemifacial twitching and progressive facial paresis (House-Brackmann grading score V/VI). The computed tomography and magnetic resonance imaging studies confirmed an expansile lesion along the vertical FN segment. Excision and histopathologic examination demonstrated FNH. FNHs involving the vertical FN segment are extremely rare. Despite being rare lesions, we believe that familiarity with the presentation and management of FNHs are imperative. Laryngoscope, 2012. Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.

A Rare Case of an Early Postoperative Obstructive Ileus in a Young Female Patient due to a Residual Trichobezoar Mass

Directory of Open Access Journals (Sweden)

P. Christopoulos

2016-01-01

Full Text Available Trichobezoar is a rare cause of small bowel obstruction, whereby a mass forms most commonly in the stomach and duodenum of young females, from ingestion of hair, a condition known as trichophagia. We present a case of recurrent small bowel obstruction due to a residual hair mass that was removed surgically in a young female patient who had a laparotomy and gastrotomy for removal of a large gastric trichobezoar just two weeks prior to the current admission. This case illustrates the importance of a thorough inspection of the whole bowel to ensure that no residual bezoars remain after surgery.

Odontoameloblastoma: A rare case with unusual presentation

Directory of Open Access Journals (Sweden)

Supreet Jain

2016-01-01

Full Text Available The odontoameloblastoma (OA, also known as ameloblastic odontoma, is a very rare odontogenic tumor that is similar to ameloblastoma in its locally aggressive behavior. OA includes odontogenic ectomesenchyme in addition to odontogenic epithelium that resembles an ameloblastoma both in structure and in behavior. Its clinical presentation, however, often mimics the more innocuous odontoma, and hence, the recognition of its aggressive nature is commonly only ascertained after its histopathologic diagnosis following enucleation. This paper presents a rare case of OA with unusual clinical and radiological features.

A rare case of Charlin's syndrome

Directory of Open Access Journals (Sweden)

Mohit P Singh

2017-01-01

Full Text Available Charlin's syndrome is an extremely rare condition characterized by pain in the nasal and paranasal areas, which is precipitated by touching the lateral aspect of the ipsilateral nostril. We are presenting one such case of a 42-year-old man who was admitted to Dr. Chaudhary Hospital and Medical Research Centre.

Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report

Science.gov (United States)

Acharya, Sonu; Panda, Swagatika; Sahoo, Sujit Ranjan; Ray, Prayas

2013-01-01

ABSTRACT Gorlin's syndrome is a rare disorder transmitted as an autosomal dominant trait. It is characterized by multiple disorders involving multiple systems. We present a case of 11-year-old male child presenting with multiple odontogenic keratocyst to the dental clinic. Retrograde diagnosis of Gorlin-Goltz syndrome was made after clinical and radiological investigation. How to cite this article: Acharya S, Panda S, Dhull KS, Sahoo SR, Ray P. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report. Int J Clin Pediatr Dent 2013;6(3):208-212. PMID:25206225

A Rare Seen Case Report: Sirenomelia

Directory of Open Access Journals (Sweden)

Nilay Piskinpasa

2016-01-01

Full Text Available Sirenomelia is a rare congenital anomaly characterized by abnormal development of the caudal body structures.The disease can be diagnosed antenatally by the careful ultrasound examination. In this paper we aimed to discuss a case which had one lower extremity,spinal angulations and anhydramnios image detected by ultrasound examination.We diagnosed sirenomelia after termination.

Illustrating, Quantifying, and Correcting for Bias in Post-hoc Analysis of Gene-Based Rare Variant Tests of Association

Science.gov (United States)

Grinde, Kelsey E.; Arbet, Jaron; Green, Alden; O'Connell, Michael; Valcarcel, Alessandra; Westra, Jason; Tintle, Nathan

2017-01-01

To date, gene-based rare variant testing approaches have focused on aggregating information across sets of variants to maximize statistical power in identifying genes showing significant association with diseases. Beyond identifying genes that are associated with diseases, the identification of causal variant(s) in those genes and estimation of their effect is crucial for planning replication studies and characterizing the genetic architecture of the locus. However, we illustrate that straightforward single-marker association statistics can suffer from substantial bias introduced by conditioning on gene-based test significance, due to the phenomenon often referred to as “winner's curse.” We illustrate the ramifications of this bias on variant effect size estimation and variant prioritization/ranking approaches, outline parameters of genetic architecture that affect this bias, and propose a bootstrap resampling method to correct for this bias. We find that our correction method significantly reduces the bias due to winner's curse (average two-fold decrease in bias, p bias and improve inference in post-hoc analysis of gene-based tests under a wide variety of genetic architectures. PMID:28959274

Rare case of transverse testicular ectopia - Case report and review of literature.

Science.gov (United States)

Raj, Vinod; Redkar, Rajeev; Krishna, Swathi; Tewari, Shruti

2017-01-01

This case report has been reported in line with the SCARE criteria; Consensus-based surgical case report guidelines of International Journal of Surgery 2016. Transverse Testicular Ectopia (TTE) is a rare condition which manifests with unilateral undescended testis and contralateral hernia. Till now around 100 cases have been described in the literature. The management depends on the anatomy of the vas, vessels and testis found on surgical exploration. An algorithm exists for its management and we propose a modified algorithm for management of TTE. Five year male presented with complaints of unilateral undescended testis on the right and hernia on the left. Clinically the right testis was impalpable and left testis palpable in the left hemiscrotum and fluid hernia on the same side. Transverse testicular ectopia is a rare condition presenting with UDT and contralateral inguinal hernia. Although more than 100 cases have been described in the literature so far, those managed with a transeptal contralateral orchidopexy are two cases to the best of our knowledge. TTE was first described by Von Lenhossek in 1886. The mean age of presentation is around 4 years and most of the cases are diagnosed on surgical exploration. The management of TTE remains controversial even though an algorithm has been described for its management due to its varied presenting scenarios. TTE is a rare condition which requires high index of suspicion for diagnosis preoperatively. Whenever suspected we recommend an USG and/or MRI prior to diagnostic laparoscopy and proceed with orchidopexy. Diagnostic laparoscopy is both helpful in diagnosis and management. Transeptal contralateral orchidopexy gives good tension free fixation of testes in the scrotum.

Invasive lobular carcinoma of the male breast - a systematic review with an illustrative case study.

Science.gov (United States)

Senger, Jenna-Lynn; Adams, Scott J; Kanthan, Rani

2017-01-01

Male breast cancer is rare, comprising only 1% of all mammary cancers; invasive ductal carcinoma is by far the commonest subtype in both men and women. Though lobular breast cancer is the second most common subtype seen in women, such cancers are extremely uncommon in men, and this is likely related to the lack of lobular development in the male breast. Thus, due to the rarity of this subtype among breast cancers, compounded by the overall rarity of breast cancer in men, current understanding of the pathogenesis of this disease and its management is largely derived from case series and extrapolation of information from the larger cohort of female patients. This paper provides a systematic review on invasive lobular carcinoma of the male breast in the context of an illustrative case study. A comprehensive analysis of the National Cancer Institute's Surveillance, Epidemiology, and End Results Data 1973-2013 leading to an exploration of the pathogenesis, epidemiology, clinical presentation, diagnosis, tumor characteristics, and management of lobular breast carcinoma in men is also discussed. Lobular subtype of breast cancer remains an enigmatic elusive disease that needs additional research to unravel its overall pathogenesis and molecular profile to provide insight for improved therapeutic management options.

Subdural haemorrhage following endoscopic third ventriculostomy. A rare complication.

LENUS (Irish Health Repository)

Kamel, M H

2012-02-03

Subdural collections or hematomas are frequently observed after shunt placement [7-9, 13], but rarely after ETV [6]. A review of literature revealed 7 cases [1, 5, 6, 10, 12], of which only 1 was symptomatic [5]. We will discuss the causes, management, and methods of prevention of this complication and we will present a case of symptomatic subdural haematoma, following endoscopic third ventriculostomy for illustration.

A Rare Case of Primary Infiltrating Neuroendocrine Carcinoma of the Breast

International Nuclear Information System (INIS)

Nawawi, Ouzreiah; Ying Goh, Keat; Rahmat, Kartini

2012-01-01

Primary neuroendocrine carcinoma of the breast is a very rare malignant tumor. There are not many cases reported in the English literature since it was first documented in 1983. Reports on the imaging features, in particular the ultrasonographic features of this rare tumor are scarce. Herein, we report a case of aggressive primary infiltrating neuroendocrine carcinoma of the breast, masquerading as an inflammatory breast condition in a 22-year-old young lady, perhaps the youngest case ever reported in the English literature. We discuss the imaging features and highlight the Doppler ultrasonographic findings of this rare breast carcinoma. This is the first documentation on Doppler ultrasonographic findings of primary neuroendocrine carcinoma of the breast in the literature

Retroperitoneal Schwannoma: A Rare Case

Directory of Open Access Journals (Sweden)

Murat Kalaycı

2011-01-01

Case Presentation. A 38-year-old woman was admitted to the emergency service with the complaints of progressive abdominal pain and nausea for the last 24 hours. Abdominal examination was compatible with acute abdomen. Acute appendicitis was diagnosed by CT. During CT evaluation, a round shaped soft-tissue mass at the retroperitoneal area inferior to the right kidney was detected, The mass was resected and histology revealed schwannoma. Conclusion. Rare tumoral lesions with benign course such as schwannoma can be detected incidentally.

BING-NEEL SYNDROME: ILLUSTRATIVE CASES AND COMPREHENSIVE REVIEW OF THE LITERATURE

Directory of Open Access Journals (Sweden)

Marzia Varettoni

2017-10-01

Full Text Available The Bing-Neel syndrome is a rare neurological complication of Waldenström’s Macroglobulinemia which results from a direct involvement of central nervous system by malignant lymphoplasmacytic cells. The clinical suspicion of Bing-Neel syndrome may be difficult because neurologic symptoms are heterogeneous, non specific and sometimes underhand. A definitive diagnosis of Bing-Neel syndrome can be confidently made using brain and spinal cord magnetic resonance imaging as well as histopathology and/or cerebrospinal fluid analysis to confirm the neoplastic infiltration of central nervous system. The detection in the cerebrospinal fluid of patients with Bing-Neel syndrome of the MYD88 (L265P somatic mutation, which is highly recurrent in Waldenström’s Macroglobulinemia, revealed useful for the diagnosis and monitoring of central nervous system involvement. Despite recommendations recently published, there is still no clear consensus on treatment of Bing-Neel syndrome, which includes systemic immunochemotherapy, intrathecal chemotherapy and brain irradiation as possible options. Ibrutinib, a Bruton kinase inhibitor highly active in patients with Waldenström’s Macroglobulinemia, has been recently added to the therapeutic armamentarium of Bing-Neel syndrome due to its ability to pass the blood-brain barrier. However, prospective clinical trials are eagerly awaited with the aim to define the optimal treatment strategy.  Here we describe four illustrative cases of Bing-Neel syndrome diagnosed and treated at our Institution and review the literature on this topic.

A rare case of adult scalp pyoderma gangrenosum with cranial osteolysis

OpenAIRE

L. Aljohmani; K. Abdul-Jalil; C. deBlacam; G.M. Murphy; J.B. O'Sullivan

2018-01-01

Pyoderma gangrenosum (PG) is a rare and painful idiopathic skin condition that has one or more areas of chronic ulceration with well demarcated and undermined borders. Bone osteolysis (the pathological destruction of bone tissue) secondary to PG is a rare phenomenon with limited cases reported in children only. This is the first case report of scalp PG with cranial osteolysis in an 80-year-old adult, with an initial presentation mimicking skin carcinoma. This case highlights the importance...

Monilethrix – Case report of a rare disease

Directory of Open Access Journals (Sweden)

Tasleem Arif

2015-01-01

Full Text Available Monilethrix is a rare genetic disorder of hair characterized by beaded appearance of the hair shaft leading to hair fragility and patchy dystrophic alopecia. In this disorder, the hair shaft has alternate widenings (nodes and constrictions (internodes that lead to fracture of hair shaft and varying degree of alopecia. We report an eight year old Kashmiri boy who presented with diffuse hair loss since infancy. As monilethrix is a rare disease entity which prompted us to report this case.

Plexiform neurofibroma causing an ossifying subperiosteal haematoma: a rare case in the tibia of an 11-year-old girl

Energy Technology Data Exchange (ETDEWEB)

Lavell, Anton [Fiona Stanley Hospital, Department of Radiology, Murdoch, WA (Australia); Jones, Christopher W. [Perth Orthopaedic Institute, Nedlands, WA (Australia); Sir Charles Gairdner Hospital, Department of Orthopaedic Surgery, Nedlands, WA (Australia); Princess Margaret Hospital, Subiaco, WA (Australia); Wong, Daniel [PathWest, QEII Medical Centre, Anatomical Pathology, Nedlands, WA (Australia); Counsel, Peter [Princess Margaret Hospital, Department of Radiology, Subiaco, WA (Australia); Perth Radiological Clinic, Mirrabooka, WA (Australia); Carey-Smith, Richard [Sir Charles Gairdner Hospital, Department of Orthopaedic Surgery, Nedlands, WA (Australia); Princess Margaret Hospital, Subiaco, WA (Australia)

2017-10-15

Ossifying subperiosteal haematoma is an exceedingly rare manifestation of Neurofibromatosis type 1 (NF-1). We report an interesting case of plexiform neurofibroma causing a rapidly growing tibial mass as a result of subperiosteal haemorrhage, in an 11-year-old girl with previously undiagnosed NF-1. This reflects a precursor of the more mature periosteal ossification seen in cases traditionally termed ''subperiosteal cysts''. A previously well young girl was referred by her general practitioner with an increasingly large, mildly tender, soft lump on the anterior aspect of her right tibia. Plain radiographs demonstrated soft tissue thickening overlying the anterior tibia, without appreciable periosteal ossification. Magnetic resonance imaging (MRI) illustrated a single central fluid-fluid level and periosteal elevation with saucerisation of the anterior tibial cortex and mild surrounding oedema. Histopathology revealed a large plexiform neurofibroma. Interestingly, this was associated with haemorrhagic change and a peripheral rim of florid reactive new bone formation. This unusual presentation was discussed at a multidisciplinary bone and soft tissue tumour meeting, where in combination with the clinical history of cafe au lait spots and positive family history, a consensus diagnosis of NF-1 was made. To date, there have only been limited case reports of this rare pathological process. In summary, this case report accounts an acute presentation of this rare osseous manifestation of NF-1, being the first to clearly demonstrate a timeline of subperiosteal haematoma with subsequent subperiosteal bone proliferation. The clinical reasoning and radiological features for such a presentation are also described. (orig.)

Plexiform neurofibroma causing an ossifying subperiosteal haematoma: a rare case in the tibia of an 11-year-old girl

International Nuclear Information System (INIS)

Lavell, Anton; Jones, Christopher W.; Wong, Daniel; Counsel, Peter; Carey-Smith, Richard

2017-01-01

Ossifying subperiosteal haematoma is an exceedingly rare manifestation of Neurofibromatosis type 1 (NF-1). We report an interesting case of plexiform neurofibroma causing a rapidly growing tibial mass as a result of subperiosteal haemorrhage, in an 11-year-old girl with previously undiagnosed NF-1. This reflects a precursor of the more mature periosteal ossification seen in cases traditionally termed ''subperiosteal cysts''. A previously well young girl was referred by her general practitioner with an increasingly large, mildly tender, soft lump on the anterior aspect of her right tibia. Plain radiographs demonstrated soft tissue thickening overlying the anterior tibia, without appreciable periosteal ossification. Magnetic resonance imaging (MRI) illustrated a single central fluid-fluid level and periosteal elevation with saucerisation of the anterior tibial cortex and mild surrounding oedema. Histopathology revealed a large plexiform neurofibroma. Interestingly, this was associated with haemorrhagic change and a peripheral rim of florid reactive new bone formation. This unusual presentation was discussed at a multidisciplinary bone and soft tissue tumour meeting, where in combination with the clinical history of cafe au lait spots and positive family history, a consensus diagnosis of NF-1 was made. To date, there have only been limited case reports of this rare pathological process. In summary, this case report accounts an acute presentation of this rare osseous manifestation of NF-1, being the first to clearly demonstrate a timeline of subperiosteal haematoma with subsequent subperiosteal bone proliferation. The clinical reasoning and radiological features for such a presentation are also described. (orig.)

On a Rare Cutaneous Metastasis from a Sacrococcygeal Chordoma

Directory of Open Access Journals (Sweden)

Alessandro D’Amuri

2017-01-01

Full Text Available Chordomas are rare malignant tumors of notochordal origin and are rare locally aggressive ones with a metastatic potential. The skin rarely is seen as metastatic site. We describe a case of an adult woman with cutaneous metastasis of a primary sacral chordoma excised ten years before, which appeared as a painless cutaneous mass located in the dorsal region. Once removed, the surgical specimen was formalin fixed and in paraffin embedded. Sections were stained with haematoxylin-eosin, and histochemical and immunohistochemical investigations were performed. Histologically, the neoplasia was characterized by cords or single tumor cells with an abundant myxoid stroma, conspicuous pale vacuolated cytoplasm (the classic “physaliphorous cells”, and mild nuclear atypia. Mitotic activity was scanty. At immunohistochemistry, the tumor cells were diffusely positive for S-100 protein, pan-keratins, EMA, and vimentin. A diagnosis of cutaneous metastasis of chordoma was performed. This case illustrates a diagnostic challenge because of the unusual presentation of an already rare tumor.

Gorlin-goltz syndrome: a rare case.

Science.gov (United States)

Ganguly, Satyaki; Jaykar, Kranti C; Kumar, Rajesh; Jha, Abhijeet Kumar; Banerjee, P K

2015-01-01

Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi.

Gorlin-Goltz Syndrome: A Rare Case

OpenAIRE

Ganguly, Satyaki; Jaykar, Kranti C; Kumar, Rajesh; Jha, Abhijeet Kumar; Banerjee, PK

2015-01-01

Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi.

Rare variant association analysis in case-parents studies by allowing for missing parental genotypes.

Science.gov (United States)

Li, Yumei; Xiang, Yang; Xu, Chao; Shen, Hui; Deng, Hongwen

2018-01-15

The development of next-generation sequencing technologies has facilitated the identification of rare variants. Family-based design is commonly used to effectively control for population admixture and substructure, which is more prominent for rare variants. Case-parents studies, as typical strategies in family-based design, are widely used in rare variant-disease association analysis. Current methods in case-parents studies are based on complete case-parents data; however, parental genotypes may be missing in case-parents trios, and removing these data may lead to a loss in statistical power. The present study focuses on testing for rare variant-disease association in case-parents study by allowing for missing parental genotypes. In this report, we extended the collapsing method for rare variant association analysis in case-parents studies to allow for missing parental genotypes, and investigated the performance of two methods by using the difference of genotypes between affected offspring and their corresponding "complements" in case-parent trios and TDT framework. Using simulations, we showed that, compared with the methods just only using complete case-parents data, the proposed strategy allowing for missing parental genotypes, or even adding unrelated affected individuals, can greatly improve the statistical power and meanwhile is not affected by population stratification. We conclude that adding case-parents data with missing parental genotypes to complete case-parents data set can greatly improve the power of our strategy for rare variant-disease association.

Granulicatella adiacens abscess: Two rare cases and review

Directory of Open Access Journals (Sweden)

Sangita Gupta

2018-01-01

Full Text Available Granulicatella adiacens is a nutritionally variant streptococcus species. These bacteria are rarely isolated in the laboratory due to their fastidious growth requirements. These have been mostly reported from bloodstream infections, infective endocarditis, infections of orbit, nasolacrimal duct and breast implants. Here, we are reporting two cases of subcutaneous abscesses caused by G. adiacens. In first case, it was isolated from abscess around elbow joint and second case was a suprapatellar abscess. We have also reviewed the published data concerning diagnosis and antimicrobial susceptibility pattern of Granulicatella infections and included some Indian cases.

Rare case of gastrointestinal stromal tumor of the anal canal

Directory of Open Access Journals (Sweden)

Madhu Kumar

2013-01-01

Full Text Available Gastrointestinal stromal tumor (GIST is a rare mesenchymal neoplasm of the gastrointestinal tract. GIST of anal canal is very rare representing only 3% of all anorectal mesenchymal tumors. We report an extremely rare case of GIST of the anal canal in 60-years-old man with history of irregular bowel habits with dark colored stool mixed with blood and constipation from 6 month. Diagnosis was made on the basis of histomorphological and immunohistochemical examination.

Invasive lobular carcinoma of the male breast – a systematic review with an illustrative case study

Science.gov (United States)

Senger, Jenna-Lynn; Adams, Scott J; Kanthan, Rani

2017-01-01

Male breast cancer is rare, comprising only 1% of all mammary cancers; invasive ductal carcinoma is by far the commonest subtype in both men and women. Though lobular breast cancer is the second most common subtype seen in women, such cancers are extremely uncommon in men, and this is likely related to the lack of lobular development in the male breast. Thus, due to the rarity of this subtype among breast cancers, compounded by the overall rarity of breast cancer in men, current understanding of the pathogenesis of this disease and its management is largely derived from case series and extrapolation of information from the larger cohort of female patients. This paper provides a systematic review on invasive lobular carcinoma of the male breast in the context of an illustrative case study. A comprehensive analysis of the National Cancer Institute’s Surveillance, Epidemiology, and End Results Data 1973–2013 leading to an exploration of the pathogenesis, epidemiology, clinical presentation, diagnosis, tumor characteristics, and management of lobular breast carcinoma in men is also discussed. Lobular subtype of breast cancer remains an enigmatic elusive disease that needs additional research to unravel its overall pathogenesis and molecular profile to provide insight for improved therapeutic management options. PMID:28553141

A Rare Case of Zosteriform Cutaneous Metastases from Squamous ...

African Journals Online (AJOL)

A Rare Case of Zosteriform Cutaneous Metastases from Squamous Cell Carcinoma of Hard Palate. ... examination, the patient had a superficial ulcer over the hard palate. A provisional diagnosis of zosteriform ... Majority of these cases can be misdiagnosed as herpes zoster and were treated with antiviral drugs. Distant ...

External auditory canal leech: a rare case report of paediatric ...

African Journals Online (AJOL)

Leeches are blood sucking organism feed on human blood. While human bites are common, they rarely cause human internal infestation. We describe a rare case of a parasitic leech infestation of the External Auditory Canal (EAC). A two month old child presented to the Emergency department with a seven day history of ...

Nodules on the Hair: A Rare Case of Mixed Piedra

OpenAIRE

Khatu, Swapna S; Poojary, Shital Amin; Nagpur, Niranjan G

2013-01-01

Piedra is a superficial fungal infection of the hair shaft characterized by nodules along the hair shaft. Black piedra affects the scalp hair more frequently than white piedra. Occurrence of both types of piedra simultaneously in a patient is extremely rare. We describe here a rare case of mixed piedra of scalp hair.

Nodules on the hair: a rare case of mixed piedra.

Science.gov (United States)

Khatu, Swapna S; Poojary, Shital Amin; Nagpur, Niranjan G

2013-10-01

Piedra is a superficial fungal infection of the hair shaft characterized by nodules along the hair shaft. Black piedra affects the scalp hair more frequently than white piedra. Occurrence of both types of piedra simultaneously in a patient is extremely rare. We describe here a rare case of mixed piedra of scalp hair.

Gorlin-Goltz syndrome: A rare case

Directory of Open Access Journals (Sweden)

Satyaki Ganguly

2015-01-01

Full Text Available Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi.

A rare case of Weil's disease with alveolar haemorrhage

Directory of Open Access Journals (Sweden)

Abhiram Chakrabarti

2014-05-01

Full Text Available Leptospirosis, a disease of protean manifestations occurs sporadically throughout the year with a peak seasonal incidence during the rainy season mimicking other febrile viral illness. In the rare case, the disease leads to renal and hepatic involvement with hemorrhage which may be associated with multisystem organ dysfunction in form of pulmonary, cardiac and central nervous system, when it is known as Weil's disease. Rarely haemorrhagic manifestations are assosciated. Early diagnosis is important as sometimes the disease may be life threatening. Proper antibiotics results in dramatic improvement. We hereby presented a case that had clinical features of Weil's disease with cough, dyspnoea and haemoptysis. Leptospirosis was detected on ELISA testing. Patient was cured rapidly with antibiotics.

A rare case of Weil's disease with alveolar haemorrhage.

Science.gov (United States)

Chakrabarti, Abhiram; Nandy, Manab; Pal, Dipankar; Mallik, Sudesna

2014-05-01

Leptospirosis, a disease of protean manifestations occurs sporadically throughout the year with a peak seasonal incidence during the rainy season mimicking other febrile viral illness. In the rare case, the disease leads to renal and hepatic involvement with hemorrhage which may be associated with multisystem organ dysfunction in form of pulmonary, cardiac and central nervous system, when it is known as Weil's disease. Rarely haemorrhagic manifestations are assosciated. Early diagnosis is important as sometimes the disease may be life threatening. Proper antibiotics results in dramatic improvement. We hereby presented a case that had clinical features of Weil's disease with cough, dyspnoea and haemoptysis. Leptospirosis was detected on ELISA testing. Patient was cured rapidly with antibiotics.

A Rare Case of β-Ketothiolase Deficiency

Directory of Open Access Journals (Sweden)

B. Modh

2015-06-01

Full Text Available We are reporting a case of β-ketothiolase deficiency, a rare disorder of amino acid metabolism. A 10 month old child presented with complaints of vomiting, convulsions, fever and altered sensorium that on investigations showed metabolic acidosis, hyperammonemia and ketosis. Gas chromatography/ mass spectroscopic examination was suggestive of β-ketothiolase deficiency.

Congenital double-double lip: A rare case report

Directory of Open Access Journals (Sweden)

Swati Phore

2017-01-01

Full Text Available A double lip is a rare anomaly characterized by a horizontal fold of redundant mucosal tissue that is situated proximal to the vermilion border. It may be either congenital or acquired and has no gender or race predilection. It occurs most often in the upper lip, although both upper and lower lips are occasionally involved. Surgical intervention (simple excision produces good functional and cosmetic results. In this report, a case of a nonsyndromic congenital maxillary double upper lip and lower lip, both are described. Double lip is of special interest in dental profession as a dental surgeon is normally the first one to diagnose this rare and uncommon condition. Authors hereby discuss a very rare condition in which both upper and lower lips are involved.

Reducing selection bias in case-control studies from rare disease registries.

Science.gov (United States)

Cole, J Alexander; Taylor, John S; Hangartner, Thomas N; Weinreb, Neal J; Mistry, Pramod K; Khan, Aneal

2011-09-12

In clinical research of rare diseases, where small patient numbers and disease heterogeneity limit study design options, registries are a valuable resource for demographic and outcome information. However, in contrast to prospective, randomized clinical trials, the observational design of registries is prone to introduce selection bias and negatively impact the validity of data analyses. The objective of the study was to demonstrate the utility of case-control matching and the risk-set method in order to control bias in data from a rare disease registry. Data from the International Collaborative Gaucher Group (ICGG) Gaucher Registry were used as an example. A case-control matching analysis using the risk-set method was conducted to identify two groups of patients with type 1 Gaucher disease in the ICGG Gaucher Registry: patients with avascular osteonecrosis (AVN) and those without AVN. The frequency distributions of gender, decade of birth, treatment status, and splenectomy status were presented for cases and controls before and after matching. Odds ratios (and 95% confidence intervals) were calculated for each variable before and after matching. The application of case-control matching methodology results in cohorts of cases (i.e., patients with AVN) and controls (i.e., patients without AVN) who have comparable distributions for four common parameters used in subject selection: gender, year of birth (age), treatment status, and splenectomy status. Matching resulted in odds ratios of approximately 1.00, indicating no bias. We demonstrated bias in case-control selection in subjects from a prototype rare disease registry and used case-control matching to minimize this bias. Therefore, this approach appears useful to study cohorts of heterogeneous patients in rare disease registries.

Fryns anophthalmia-plus syndrome: two rare cases.

Science.gov (United States)

Bozkurt, O; Bidev, D; Sari, F N; Dizdar, E A; Ulu, H O; Uras, N; Oguz, S S; Canpolat, F E; Dilmen, U

2014-01-01

Fryns anophthalmia-plus syndrome is a rare syndrome with clinical diversity primarily including anophthalmia/microphthalmia, facial clefts, cleft lip/palate, ear and nasal deformities. Here we present two different cases of APS with anopthalmia/microphthalmia, cleft palate, low set ears, ventriculomegaly and one of which had intestinal non-fixation anomaly not described in the literature before.

Ellis–Van Creveld syndrome in siblings: A rare case report

Directory of Open Access Journals (Sweden)

Sabitha Gokulraj

2016-01-01

Full Text Available Ellis–Van Creveld syndrome or chondroectodermal dysplasia is a rare autosomal recessive disorder presenting several skeletal manifestations and congenital heart malformations. Ellis–Van Creveld syndrome comprises of a tetrad of clinical manifestations of chondrodysplasia, polydactyly, ectodermal dysplasia, and cardiac defects. Here, we are presenting a very rare case of Ellis–Van Creveld syndrome in siblings.

Rare Case of Polymicrobial Keratitis With Balantidium coli.

Science.gov (United States)

Hazarika, Manali; Pai H, Vijaya; Khanna, Vinay; Reddy, Harish; Tilak, Kriti; Chawla, Kiran

2016-12-01

To report a rare case of polymicrobial keratitis due to Balantidium coli and gram-negative bacteria, Pseudomonas aeruginosa and Klebsiella pneumoniae, in a soft contact lens (CL) wearer. We report a case of CL-related keratitis due to B. coli, P. aeruginosa, and K. pneumoniae. The culture of the corneal scrapings, the CL cleaning solution, and the CL revealed the growth of a rare ciliated parasite, B. coli, along with gram-negative bacteria, namely, P. aeruginosa and K. pneumoniae. The patient was successfully treated with topical broad-spectrum antibiotics and intravenous metronidazole. Polymicrobial keratitis has seldom been reported with B. coli as the causative agent. CL wear can be a risk factor for this infection. Treatment with topical antibiotics may not suffice, and the intravenous route of antiprotozoal drugs may be a useful adjunct. Increasing awareness, early diagnosis, and treatment may improve the final visual outcome.

Dipylidium caninum infection in a child: A rare case report

Directory of Open Access Journals (Sweden)

M V Narasimham

2013-01-01

Full Text Available Dipylidiasis is a zoonotic parasitic infestation caused by the dog tapeworm Dipylidium caninum. Human dipylidiasis has been rarely reported in English literature. Young children are mostly at risk of acquiring the infection due to their close association with dogs and cats. We report a rare case of Dipylidium caninum infection in a 4 year old male child. The diagnosis was based on microscopic examination of stool. Confirmation of the proglottid segments was done by histopathological examination. To the best of our knowledge this is the first human case of Dipylidium caninum reported from this part of the country.

Dipylidium caninum infection in a child: a rare case report.

Science.gov (United States)

Narasimham, M V; Panda, P; Mohanty, I; Sahu, S; Padhi, S; Dash, M

2013-01-01

Dipylidiasis is a zoonotic parasitic infestation caused by the dog tapeworm Dipylidium caninum. Human dipylidiasis has been rarely reported in English literature. Young children are mostly at risk of acquiring the infection due to their close association with dogs and cats. We report a rare case of Dipylidium caninum infection in a 4 year old male child. The diagnosis was based on microscopic examination of stool. Confirmation of the proglottid segments was done by histopathological examination. To the best of our knowledge this is the first human case of Dipylidium caninum reported from this part of the country.

Rare Case of Duodenal Metastasis From Pulmonary Squamous Cell Carcinoma

Directory of Open Access Journals (Sweden)

Zain Memon DO

2017-10-01

Full Text Available Pulmonary squamous cell carcinoma is the second most common non–small cell malignancy of the lung. It commonly metastasizes to the adrenal glands, bone, liver, brain, and kidneys. Most occurrences of metastatic squamous cell carcinoma involving the gastrointestinal tract originate from primary lung tumors. Metastasis to the duodenum, however, is exceedingly rare, with very few cases of stomach or duodenal involvement described in the literature. We report the case of a patient with stage IV pulmonary squamous cell carcinoma metastasizing to the duodenum with an uncommon presentation to add to the paucity of literature available regarding this rare finding.

Sirenomelia and ndash; Mermaid Syndrome with Oesophageal Atresia: A Rare Case Report

Directory of Open Access Journals (Sweden)

Veena Raja

2015-09-01

Full Text Available Sirenomelia or Mermaid syndrome is a very rare congenital deformity in which legs are fused together and commonly associated with abnormal kidney development, genital, and rectal abnormalities. In this present case, sirenomelia was associated with oesophageal atresia, which is a rare association and occurs in about 20-35% of cases. [J Interdiscipl Histopathol 2015; 3(3.000: 113-116

ASPERGILLOSIS OF MANDIBLE : A RARE CASE OF OSTEOMYELITIS

OpenAIRE

ÖZTÜRK, Nurdan; ERSOY, Burak; SÖNMEZ, Ahmet; ÇELEBİLER, Özhan; NUMANOĞLU, Ayhan

2007-01-01

Aspergillus species are saprophyticus fungi which may be the cause of infection predominantly in immunocompromised hosts. Aspergillosis is usually manifested in the respiratory system and bone involvement is rarely encountered. Osteomyelitis of the mandible with aspergillus species has been reported in only two cases in the literature. Treatment mainly consists of antimicrobial therapy in combination with surgery. We report a case of aspergillosis of the mandible in a patient who was under im...

Sturge Weber Syndrome: review of literature with case illustration

Directory of Open Access Journals (Sweden)

Satyarthee Guru Dutta

2017-03-01

Full Text Available Sturge-Weber syndrome (SWS also called as encephalotrigeminal angiomatosis, is a sporadically occurring rare neuro-cutaneous syndrome, characterized by vascular malformation with capillary venous angiomas involving face, choroidal layer of eye globe and leptomeninges responsible for ophthamological as well as neurological signs and symptoms. Authors report an interesting case, a six year old girl, who presented with seizures, facial port wine stain and normal psychomotor development. CT scan showed left cerebral hemiatrophy, left frontal and parieto occipital calcification with cortical calcification in left high frontal convexity. Cranial MRI scan also confirmed finding of left cerebral hemiatrophy and also revealed presence of gyriform cortical calcification, prominent flow voids seen in left basal ganglia. Her seizure is well controlled with antiepileptic medication. The pertinent literature is reviewed and management of such cases is discussed briefly.

Sirenomelia sequence: early prenatal diagnosis of one rare case associated with acardiac malformation.

Science.gov (United States)

Zanforlin Filho, Sebastião M; Guimarães Filho, Hélio A; Araujo Júnior, Edward; Pires, Cláudio R; Mattar, Rosiane; Nardozza, Luciano M M

2007-04-01

Sirenomelia sequence is a very rare congenital malformation, with incidence of around 1.5-4.2 per 100,000 births. Prenatal diagnosis of sirenomelia in the first trimester is rare; there are only five cases reported for the present, and the association of sirenomelia with acardiac malformation is even rarer. We present a rare case of sirenomelia associated with acardiac malformation detected in the first trimester through combined two-dimensional, three-dimensional and color Doppler sonographies.

A rare case of juvenile dermatomyositis and review of literature

Directory of Open Access Journals (Sweden)

Anjali T Bharani

2017-01-01

Full Text Available Idiopathic inflammatory myopathies are rare group of systemic connective tissue diseases. The hallmark of these disorders is symmetrical chronic inflammation and weakness of proximal muscles. Juvenile dermatomyositis (JDM is the most common inflammatory myositis in children. We describe a rare case of JDM in a 4-year-old female child who presented with characteristic cutaneous rash and proximal muscle weakness.

Focal dermal hypoplasia: A rare case report

Directory of Open Access Journals (Sweden)

Sahana M Srinivas

2015-01-01

Full Text Available Focal dermal hypoplasia (Goltz syndrome is a rare genetic multisystem disorder primarily involving the skin, skeletal system, eyes, and face. We report the case of an eight-month-old female child who presented with multiple hypopigmented atrophic macules along the lines of blaschko, skeletal anomalies, umbilical hernia, developmental delay, hypoplastic nails, syndactyly, and lobster claw deformity characteristic of Goltz syndrome.

A Rare Case of Petrified Ear

Directory of Open Access Journals (Sweden)

Kathryn E. Buikema

2012-01-01

Full Text Available Calcification or ossification of the auricle, also referred to as petrified ear, is a rare diagnosis in dermatology. In medical literature, it has most often been attributed to trauma, hypothermia and frostbite, or hypercalcemia secondary to a metabolic or endocrine disorder, such as Addison's disease. Here, we report the clinical and radiologic findings of a 79-year-old African American male whose unilateral petrified auricle was an incidental finding. He had a preceding history of hyperparathyroidism and subsequent hypercalcemia treated with a subtotal parathyroidectomy three years prior to presentation. In addition to laboratory analysis, a history and physical examination was performed which revealed no other signs of hypercalcemia. Radiologic studies demonstrated partial ossification of the external auricular cartilage on the left side. The patient was diagnosed with the rare occurrence of a petrified ear. In light of this case, we provide a discussion concerning the possible etiologies of this diagnosis including appropriate patient evaluation and possible treatment recommendations.

Oral lymphangioma: A rare case report

Directory of Open Access Journals (Sweden)

Harsha Bhayya

2015-01-01

Full Text Available Lymphangiomas are benign hamartomatous tumors of the lymphatic channels which present as developmental malformations arising from sequestration of lymphatic tissue that do not communicate with the rest of the lymphatic channels. Lymphatic vessels are filled with a clear protein-rich fluid containing few lymph cells. It can also occur in association with hemangioma. The onset of lymphangiomas are either at birth (60% to 70% or up to two years of age (90% and rare in adults. Lymphangiomas have marked predilection for the head and neck region (50-70%. The most common location in the mouth is the dorsum of tongue, followed by lips, buccal mucosa, soft palate, and floor of the mouth. On tongue, they may present as a localized or a diffused growth which may enlarge to cause macroglossia, impaired speech and difficulty in mastication. Herewith, we present a rare case of lymphangioma of tongue leading to macroglossia in a 8-year-old boy.

Reducing selection bias in case-control studies from rare disease registries

Directory of Open Access Journals (Sweden)

Mistry Pramod K

2011-09-01

Full Text Available Abstract Background In clinical research of rare diseases, where small patient numbers and disease heterogeneity limit study design options, registries are a valuable resource for demographic and outcome information. However, in contrast to prospective, randomized clinical trials, the observational design of registries is prone to introduce selection bias and negatively impact the validity of data analyses. The objective of the study was to demonstrate the utility of case-control matching and the risk-set method in order to control bias in data from a rare disease registry. Data from the International Collaborative Gaucher Group (ICGG Gaucher Registry were used as an example. Methods A case-control matching analysis using the risk-set method was conducted to identify two groups of patients with type 1 Gaucher disease in the ICGG Gaucher Registry: patients with avascular osteonecrosis (AVN and those without AVN. The frequency distributions of gender, decade of birth, treatment status, and splenectomy status were presented for cases and controls before and after matching. Odds ratios (and 95% confidence intervals were calculated for each variable before and after matching. Results The application of case-control matching methodology results in cohorts of cases (i.e., patients with AVN and controls (i.e., patients without AVN who have comparable distributions for four common parameters used in subject selection: gender, year of birth (age, treatment status, and splenectomy status. Matching resulted in odds ratios of approximately 1.00, indicating no bias. Conclusions We demonstrated bias in case-control selection in subjects from a prototype rare disease registry and used case-control matching to minimize this bias. Therefore, this approach appears useful to study cohorts of heterogeneous patients in rare disease registries.

Mesenchymal Chondrosarcoma of Maxilla: A Rare Case Report and Review of Literature

Directory of Open Access Journals (Sweden)

R Thriveni

2010-01-01

Full Text Available Mesenchymal condrosarcomas are rare malignant neoplasms that can arise from both soft and hard tissues. They are distinct tumors arising in unicentric or multicentric locations. They reveal unusual clinical behavior, characteristic histopathological features, and poor prognosis with late recurrences. Here is a case report of a rare case of mesenchymal chondrosarcoma arising in a 19-year-old female patient′s right maxilla.

Unusual clinical presentation of rare case of vaginal leiomyoma: a case report

OpenAIRE

Ishrat Zuber; Purnima K. Nadkarni; Aditi A. Nadkarni; Akshay Nadkarni

2016-01-01

Primary vaginal leiomyoma are rare and usually arise from anterior vaginal wall, approximately 330 cases of vaginal fibroid reported in world literature. Vaginal myoma usually presented as discharge per vaginum, abnormal bleeding, pain lower abdomen, dyspareunia etc. We report a case of primary vaginal leiomyoma arising from lateral vaginal wall which is presented clinically as pain in hip joint and radiating to ipsilateral leg which is unusual clinical presentation creating diagnostic dilemm...

Spontaneous knot; a rare cause of ventriculoperitoneal shunt blockage.

LENUS (Irish Health Repository)

Mohammed, Wail

2011-02-01

A 14-year old X linked congenital hydrocephalus presented with unexplained headaches and vomiting. He had external ventricular drain and intracranial pressure monitoring (ICP). Subsequently, he underwent exploration and removal of previously inserted ventriculoperitoneal (VP) shunts. On retrieval of peritoneal catheters a double knot was noted between his two distal catheters. This case illustrates a rare cause of ventriculoperitoneal shunt malfunction.

Spontaneous knot; a rare cause of ventriculoperitoneal shunt blockage.

LENUS (Irish Health Repository)

Mohammed, Wail

2012-02-01

A 14-year old X linked congenital hydrocephalus presented with unexplained headaches and vomiting. He had external ventricular drain and intracranial pressure monitoring (ICP). Subsequently, he underwent exploration and removal of previously inserted ventriculoperitoneal (VP) shunts. On retrieval of peritoneal catheters a double knot was noted between his two distal catheters. This case illustrates a rare cause of ventriculoperitoneal shunt malfunction.

A rare case of spinal dural arteriovenous fistula

Directory of Open Access Journals (Sweden)

Mariya Apostolova

2012-12-01

Full Text Available Spinal dural arteriovenous fistula (SDAVF is a rare vascular malformation of the spine. Only a limited number of cases of SDAVF have been reported in the current literature. We describe the case of a 74 year old male who presented with gradually progressive bilateral lower extremity weakness and bladder dysfunction and was subsequently diagnosed with SDAVF affecting both the thoracic and lumbar spine. The patient later underwent embolization with some improvement in his neurologic symptoms.

Myxofibrosarcoma of maxilla: A case report of rare entity

Directory of Open Access Journals (Sweden)

Venkateswarlu Nallapu

2015-01-01

Full Text Available Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients that rarely affect the oral cavity. In this article we describe a case of primary myxofibrosarcoma of maxilla in a 19-year-old boy. Microscopically the tumor showed loosely arranged spindle-shaped tumor cells with mild pleomorphic spindle-shaped nuclei, some with minute nucleoli in a fibromyxoid stroma. The cells were strongly immunoreactive for vimentin and negative for CD-68, S-100 protein and desmin. Based on clinical, histological and immunohistochemical findings, a final diagnosis of myxofibrosarcoma was established. It is an unusual aggressive variant that frequently recurs and metastasizes, reinforcing the importance of its correct diagnosis. We report a rare case of myxofibrosarcoma in maxilla, with emphasis on immunohistochemical findings.

Rare case of primary spinal ependymomatosis occurring in a 26-year-old man: a case report.

LENUS (Irish Health Repository)

Kaliaperumal, Chandrasekaran

2009-01-01

The authors report a rare case of primary spinal ependymomatosis in a young adult man. Multiple primary ependymomatous lesions were seen on magnetic resonance imaging and no anaplasia was identified on the surgical-pathological analysis. The aetio-pathological mechanism and surgical significance of this rare occurrence is discussed.

A Rare Case of Craniopharyngioma in the Temporal Lobe

Directory of Open Access Journals (Sweden)

Sasan Razmjoo

2017-01-01

Full Text Available Herein, we report on a rare case of craniopharyngioma arising in the left temporal lobe with no prior history of head trauma or surgery. There was a solid-cystic mass in the left temporal lobe on MR images. To the best of our knowledge, this is the second case of a craniopharyngioma occurring in the temporal lobe.

Orthognathic Surgery: Planning and treatment with illustration on six cases

International Nuclear Information System (INIS)

AiRuhaimi, K; Nwoku, A. L; Shaikh, H. S

1991-01-01

Almost all conferences for plastic and maxillofacial surgery discuss reports on several methods of orthognathic surgery, planning, success results, and complications of the different procedures carried out to correct patient's soft and hard tissues frontal profiles and occlusal discrepancies. Various principles are involved in the diagnosis and treatment of facial deformities. However, the most important consideration, after all, is the final accepted aesthetic and functional requirements and stability of the moved segments. The objective of this paper is to give the basic principles of treatment planning for correcting facial discrepancies, surgical approach to different cases, and the methods to increase stability of the moved segments. Six cases are included to illustrate the different aspects of treatment planning, surgical management, and stabilization methods. (author)

Seckel syndrome: A rare case report

Directory of Open Access Journals (Sweden)

Rinky Sisodia

2014-01-01

Full Text Available Seckel syndrome (SS is a rare, autosomal recessive syndrome; characterized by severe intrauterine and postnatal growth retardation, microcephaly, mental retardation, and typical facial appearance with beaklike protrusion of the midface (bird headed. In addition to the characteristic craniofacial dysmorphism and skeletal defects, abnormalities have been described in the cardiovascular, hematopoietic, endocrine, gastrointestinal, and central nervous systems. Usually such patients have poor psychomotor development. This case report presents an 8-year-old child with SS born to parents, exposed in Bhopal gas disaster.

A Rare Case of Aneurysmal Bone Cyst in the Paranasal Sinus

Directory of Open Access Journals (Sweden)

Seyyed Mostafa Hashemi

2015-09-01

Full Text Available Introduction: Aneurysmal Bone cysts (ABC are extremely rare in the head and neck region and even rarer in sinuses.  ABC is a benign multicystic mass that is locally-destructive and rapidly expandable. Hemorrhagic fluid content (like in this case and septated appearance are the characteristic feature of ABC. Established treatment options for ABCs include sclerotherapy, embolization, radiotherapy, simple curettage, surgical excision, or a combination of methods.   Case Report: In this article, a 5 year-old boy with a recurrent nasal mass is presented. The patient was finally diagnosed with this rare entity: ABC of the paranasal sinuses. The patient was treated through complete surgical removal.   Conclusion:  ABC can be considered as a rare differential diagnosis of recurrent nasal hemorrhagic mass in a pediatric population.

Rare vertebral metastasis in a case of Hereditary Paraganglioma

Directory of Open Access Journals (Sweden)

da Silva Manuel Eduardo

2012-09-01

Full Text Available Abstract Paragangliomas are rare tumours with a prevalence of 1/10000 to 1/30000. Tumors arising from the paraganglia are characteristically of low malignant potential. Vertebral metastases are exceedingly rare, and only isolated case reports have described them. The authors present the clinical course of a 47 years-old female patient with a familial paraganglioma [PGL] with vertebral metastastization, who underwent an intralesional tumor excision and corpectomy. Genetic screening demonstrated a new germinal frameshift mutation of the SDHB exon 6 [c.587-591DelC]. After surgery there was normalization of the analytical parameters and imagiologic screening. One year later she presented a new image in the the pedicle of T11 on the contralateral side of the surgical incision. She performed 2 treatments with MIBG and 1 cicle of radiotherapy that made the new lesion regress. Currently the patient does not present any clinical or analytical evidence of new metastasis. This case outlines the clinical course of a patient with a PGL syndrome for whom a rare vertebral metastasis was diagnosed. It highlights the importance of identifying patients with germline SDHB mutations, as these patients are at a high risk of developing malignant disease.

KIKUCHI-FUJIMOTO DISEASE (KFD): A Rare Case Report

OpenAIRE

Dr. Gunja Jain; Dr. Mayank Gupta; Dr. Laxmikant Goyal; Dr. Jai Purohit; Dr. Sudhir Mehta

2017-01-01

Abstract— Kikuchi-Fujimoto disease (KFD) is a rare disease. It has a worldwide distribution with a higher prevalence in Asians. KFD is usually a self limiting disease and benign in nature. Clinically it presents as regional cervical lymphadenopathy and sometimes may presents as generalized lymphadenopathy as well. Night sweats and low grade fever may also be associated in some cases. A case of a 36 year old female had attended in SMS Hospital. She presented with fever, weight loss and tender ...

Hidradenocarcinoma eccrinale syringomatodes of the eyelid--case of a rare malignancy.

Science.gov (United States)

Karolina, Ziaja; Arkadiusz, Pogrzebielski; Jolanta, Orłowska-Heitzman; Bozena, Romanowska-Dixon

2010-01-01

Presentation of a case of very rare malignant tumor of eccrine sweat glands in the eyelid. A 44 years old man with a tumor of the upper left eyelid is presented. Sweat gland carcinomas are rare malignant tumors of the skin adnexa. Diagnosis of these carcinomas is difficult due to their infrequency.

A Rare Case of Second Trimester Uterine Rupture in Unscarred Uterus

Directory of Open Access Journals (Sweden)

Poonam Kashyap

2017-11-01

Full Text Available Second trimester uterine rupture is rare and presents with life threatening emergency. Few cases have been reported in literature. Most of the cases which have been reported are with the use of labor-induction agents in late termination of pregnancy. Others are involving any surgical procedures in placenta percreta or scarred uterus. The main contributory factors were scarred uterus and abnormal placentation. In this study, uterine rupture had occurred in unscarred uterus in non-laboring women in second trimester without any intervention which is very rare to found. The lack of index of high suspicion diverts attention to search for other non-gynecological causes. Our aim is to create awareness through this case study to avoid delay in diagnosis and timely management of such cases.

Malignant mandibular tumors: two case reports of rare mandibular ...

African Journals Online (AJOL)

Arun Kumar Agnihotri

2014-02-26

Feb 26, 2014 ... present two cases of rare malignant mandibular tumors in a single institution. KEY WORDS: .... Spiculated osteoblastic periosteal reaction was noted with mild ... displacement of right 2nd premolar and 2nd molar teeth were ...

Galactosemia: a rare case in pediatric practice

Directory of Open Access Journals (Sweden)

S. Ya. Volgina

2015-01-01

Full Text Available Galactosemia is a rare life-threatening inherited autosomal recessive disease, the differential diagnosis of which has been very difficult up to date, especially if there are no results of neonatal screening for some reasons. The paper describes a clinical case of a 10-day patient with type I classic galactosemia and reflects the importance of a timely diagnostic search and switching him to lactose-free formulas.

Rare manifestations of sirenomelia syndrome: a report of five cases.

Science.gov (United States)

Drossou-Agakidou, Vasiliki; Xatzisevastou-Loukidou, Chariklia; Soubasi, Vasiliki; Kostopoulou, Eyanthia; Laporda, Alexandra; Pantzaki, Afroditi; Agelidou, Stamatia; Kremenopoulos, Georgios

2004-10-01

Five cases of sirenomelia presented with rare manifestations are discussed. Three neonates were born alive and died within 2 to 12 hours after birth. One case was the offspring of a triple in vitro fertilization pregnancy with history of early intrauterine death of one of the triplets. The main features included fusion of lower extremities (five of five), renal agenesis (three of five), polycystic renal dysplasia (two of five), anal atresia with large bowel hypoplasia (three of five), pulmonary hypoplasia (four of five), and single umbilical artery (five of five). Other features that have only rarely been associated with sirenomelia included concurrence of congenital heart disease and neuroblastoma, gallbladder agenesis, and upper extremity defects.

Management of A Rare Case of Communicating Internal-External Inflammatory Resorption.

Science.gov (United States)

Arora, Suraj; Gill, Gurdeep Singh; Saluja, Priyanka; Setia, Vikas

2015-05-01

The present case describes the successful management of a rare case of communicating internal-external resorption in which both internal and external resorption seem to develop independent of each other. The case report highlights the importance of correct diagnosis and need of revision of classification system of resorptive defects.

Radiology illustrated. Gastrointestinal tract

International Nuclear Information System (INIS)

Choi, Byung Ihn

2015-01-01

Radiology Illustrated: Gastrointestinal Tract is the second of two volumes designed to provide clear and practical guidance on the diagnostic imaging of abdominal diseases. The book presents approximately 300 cases with 1500 carefully selected and categorized illustrations of gastrointestinal tract diseases, along with key text messages and tables that will help the reader easily to recall the relevant images as an aid to differential diagnosis., Essential points are summarized at the end of each text message to facilitate rapid review and learning. Additionally, brief descriptions of each clinical problem are provided, followed by case studies of both common and uncommon pathologies that illustrate the roles of the different imaging modalities, including ultrasound, radiography, computed tomography, and magnetic resonance imaging.

Radiology illustrated. Gastrointestinal tract

Energy Technology Data Exchange (ETDEWEB)

Choi, Byung Ihn (ed.) [Seoul National University Hospital (Korea, Republic of). Dept. of Radiology

2015-02-01

Radiology Illustrated: Gastrointestinal Tract is the second of two volumes designed to provide clear and practical guidance on the diagnostic imaging of abdominal diseases. The book presents approximately 300 cases with 1500 carefully selected and categorized illustrations of gastrointestinal tract diseases, along with key text messages and tables that will help the reader easily to recall the relevant images as an aid to differential diagnosis., Essential points are summarized at the end of each text message to facilitate rapid review and learning. Additionally, brief descriptions of each clinical problem are provided, followed by case studies of both common and uncommon pathologies that illustrate the roles of the different imaging modalities, including ultrasound, radiography, computed tomography, and magnetic resonance imaging.

Primary Renal Synovial Sarcoma: A Rare Case Report

Directory of Open Access Journals (Sweden)

Taha Numan Yıkılmaz

2016-12-01

Full Text Available Synovial sarcoma (SS is mainly derived from soft tissues. Primary renal SS is a very rare malignancy with around 60 cases reported in the literature. We report a renal mass which was undistinguishable from urothelial carcinoma clinically and pathologically but diagnosed as a primary renal SS at the definitive pathological diagnosis.

Myoepithelial carcinoma of the male breast: a rare case report ...

African Journals Online (AJOL)

Myoepithelial carcinoma (malignant myoepithelioma) of the breast is a rare entity and in the male breast it is even rarer. Two cases of benign myoepithelioma in the male breast have been reported so far. Here we report, probably the first case of Myoepithelial carcinoma in a male breast with clinical features mimicking ...

Synovial Sarcoma of the Buccal Mucosa: A Rare Case Report

Directory of Open Access Journals (Sweden)

Kumar T. S. Mahesh

2013-01-01

Full Text Available Synovial sarcoma (SS is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Synovial sarcoma is a malignant soft tissue tumor representing 5.6% to 10% of all soft tissue sarcomas. They are termed SS because of their histologic resemblance to the synovium, but they rarely involve a synovial structure and are thought to arise from pluripotential mesenchymal cells. The tumor usually occurs in close association with tendon sheaths, bursae, and joint capsules, primarily in the para-articular regions of the extremities, with approximately 9% occurring in the head and neck region. Synovial sarcoma has been reported rarely in the oral cavity. We report a very rare case of Synovial sarcoma of the buccal mucosa in a 24-year-old male patient.

Rare Case of Vasculitis of the Hepatic Artery.

Science.gov (United States)

Mali, Padmavathi; Muduganti, Sudheer R; Goldberg, Jerry

2015-12-01

Vasculitis is an accumulation of inflammatory leucocytes in the blood vessels with reactive damage to mural structures. Isolated vasculitis of the gastrointestinal tract without systemic involvement is rare. We report a unique case of a female patient who presented with abdominal pain, and was found, on serology, to have elevated inflammatory markers without autoantibodies. A computed tomography scan of the abdomen and pelvis was suggestive of vasculitis of the hepatic artery. To the best of our knowledge, this is the first case, to date, of vasculitis of hepatic artery. © 2015 Marshfield Clinic.

A RARE CASE OF THIAZIDE-INDUCED BENIGN GYNAECOMASTIA

Directory of Open Access Journals (Sweden)

Sandeep Yamsani

2017-02-01

Full Text Available BACKGROUND Gynaecomastia is a common disorder of the endocrine system in which there is a noncancerous enlargement of the male breast. The incidence of drug-induced gynaecomastia is about 4-10%. One among them are the thiazide group of diuretics, which result in gynaecomastia by inhibition of androgen synthesis. The present case study is about a 75-year-old patient who is a known case of diabetes and hypertension with primary complaints of bilateral breast enlargement diagnosed with a rare presentation of hydrochlorothiazide adverse drug reaction gynaecomastia.¹

A rare presentation in two cases with review of literature

African Journals Online (AJOL)

2011-07-29

Jul 29, 2011 ... liver, lungs, and the eyes.[1] Lymphadenopathy is a rare mode of presentation of cysticercus infestation.[2,3] We report two cases of cysticercus lymphadenitis involving the cervical lymph nodes. Case Report. A 7-year-old male child and a 28-year-old woman, both vegetarians by their food habit, presented ...

Dipylidium caninum infection in a child: A rare case report

OpenAIRE

M V Narasimham; P Panda; I Mohanty; S Sahu; S Padhi; M Dash

2013-01-01

Dipylidiasis is a zoonotic parasitic infestation caused by the dog tapeworm Dipylidium caninum. Human dipylidiasis has been rarely reported in English literature. Young children are mostly at risk of acquiring the infection due to their close association with dogs and cats. We report a rare case of Dipylidium caninum infection in a 4 year old male child. The diagnosis was based on microscopic examination of stool. Confirmation of the proglottid segments was done by histopathological examinati...

A rare case of thyroid metastasis from pancreatic adenocarcinoma.

LENUS (Irish Health Repository)

Kelly, Michael E

2012-02-01

CONTEXT: Thyroid metastasis from pancreatic adenocarcinoma is extremely rare, with only two previous cases in the literature. We report a case of pancreatic adenocarcinoma metastasising to the thyroid. We review the incidence, diagnosis, and management of this rare occurrence. CASE REPORT: A 38-year-old man with a synchronous 6-month history of thyroid swelling, presented with epigastric pain and signs of obstructive jaundice. He was investigated by abdominal computerised tomography and endoscopic retrograde cholangiopancreatography. The diagnosis of pancreatic neoplasm was made. His thyroid neoplasm was investigated at another tertiary centre and thought to be a papillary neoplasm. He underwent a pancreaticoduodenectomy and recovered well post-operatively. Eight weeks later he had a total thyroidectomy. Histology confirmed that the thyroid mass was both morphologically and immunophenotypically similar to the pancreatic neoplasm. CONCLUSION: This case demonstrates the importance of a full investigation when a patient with suspected neoplastic history presents with a thyroid nodule. We outline the crucial role that immunohistochemistry plays in detecting and classifying primary and secondary thyroid neoplasms. The detection of a solitary thyroid metastasis from pancreatic adenocarcinoma may indicate a poor prognosis, and it is debatable whether resection of the primary should be undertaken when it presents with a solitary metastasis.

Gingival enlargement unveiling sarcoidosis: Report of a rare case

Directory of Open Access Journals (Sweden)

Sabeeha Abbas Kadiwala

2013-01-01

Full Text Available Sarcoidosis is classified as an acquired systemic granulomatous disease. Because of the fact that sarcoidosis affects multiple tissues and organs, it is characterized by many potential signs and symptoms, as well as by the presence of non-caseating granulomas in the organs involved. Although oral sarcoidosis is relatively rare, it may however, present in the oral cavity. This report presents a rare case of sarcoidosis with the initial presenting symptom as severe generalized gingival enlargement. The gingival enlargement was treated by gingivectomy. After histopathological examination of gingival biopsy and certain special investigations, a diagnosis of sarcoidosis was made.

Familial gingival fibromatosis: A rare case report

Directory of Open Access Journals (Sweden)

Shweta Sharma

2012-01-01

Full Text Available Hereditary gingival fibromatosis is a rare condition that can occur as an isolated disease or as part of a syndrome or chromosomal abnormality. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe functional and aesthetic concerns. Here, we present a case of an 8-year-old girl with severe enlargement of gums in maxilla and mandible. Both deciduous and permanent teeth were not erupted in the oral cavity at all. Mutation in the Son-of-Sevenless (SOS-1 gene has been associated with the disease. The diagnosis was made based on clinical examination and family history. Surgical removal of the hyperplastic tissue was performed under general anesthesia.

HARLEQUIN ICTHYOSIS: A RARE CASE REPORT

Directory of Open Access Journals (Sweden)

Pradipprava Paria

2016-01-01

Full Text Available Harlequin ichthyosis is the most severe form of congenital ichthyosis. It is a rare autosomal recessive disorder (1:300,000. The vast majority of affected individuals are due to mutation in the ABCA12 gene, which cause a deficiency of the epidermal lipid transporter, resulting in hyperkeratosis and abnormal barrier function of skin. Infants are very susceptible to metabolic abnormalitites and infections. They usually do not survive for very long. we report here a case of a new born with harlequin ichthyosis of consaguinious parentage who had a history of similar birth previously.

Benedikt's Syndrome: A Rare Case Report

Directory of Open Access Journals (Sweden)

Aslı Aksoy Gundogdu

2017-08-01

Full Text Available Benedikt syndrome is a rare midbrain syndrome which is associated with the damage of the median mesencephalic tegmentum. The most common etiology of this syndrome is ischemic stroke. The occlusion of the posterior cerebral artery or the paramedian branches of the basilar artery results with the ischemia of this midbrain territory. Ipsilateral occulomotor cranial nerve palsy, contralateral hemiparesis, hemihypoesthesia, hemiataxia and korea or tremor are the clinical symptoms of this syndrome. In this article, we reported a case of Benedikt syndrome with an etiologic cause of cardioembolic stroke, who was diagnosed by the neurological examination and neuroradiological findings.

Isolated pulmonary candidiasis in a patient with diabetes mellitus: A rare case report

OpenAIRE

Hakamifard, Atousa; Khorvash, Farzin; Raisi, Arash

2016-01-01

Fungal infections are as a cause of morbidity and mortality in immunocompromise patients. Because the respiratory tract is colonized with Candida, the presence of this agent in respiratory specimens makes the diagnosis of Candida pneumonia problematic. Candida pneumonia is a rare infection, and the majority of cases are secondary to hematogenous dissemination. Furthermore isolated Candida pneumonia originating from endotracheal inoculation is an extremely rare entity. We describe a case of is...

Rare causes of scoliosis and spine deformity: experience and particular features

Directory of Open Access Journals (Sweden)

Pliarchopoulou Fani M

2007-10-01

Full Text Available Abstract Background Spine deformity can be idiopathic (more than 80% of cases, neuromuscular, congenital or neurofibromatosis-related. However, there are many disorders that may also be involved. We present our experience treating patients with scoliosis or other spine deformities related to rare clinical entities. Methods A retrospective study of the records of a school-screening study in North-West Greece was performed, covering a 10-year period (1992–2002. The records were searched for patients with deformities related to rare disorders. These patients were reviewed as regards to characteristics of underlying disorder and spine deformity, treatment and results, complications, intraoperative and anaesthesiologic difficulties particular to each case. Results In 13 cases, the spine deformity presented in relation to rare disorders. The underlying disorder was rare neurological disease in 2 cases (Rett syndrome, progressive hemidystonia, muscular disorders (facioscapulohumeral muscular dystrophy, arthrogryposis in 2 patients, osteogenesis imperfecta in 2 cases, Marfan syndrome, osteopetrosis tarda, spondyloepiphyseal dysplasia congenita, cleidocranial dysplasia and Noonan syndrome in 1 case each. In 2 cases scoliosis was related to other congenital anomalies (phocomelia, blindness. Nine of these patients were surgically treated. Surgery was avoided in 3 patients. Conclusion This study illustrates the fact that different disorders are related with curves with different characteristics, different accompanying problems and possible complications. Investigation and understanding of the underlying pathology is an essential part of the clinical evaluation and preoperative work-up, as clinical experience at any specific center is limited.

'Doctor Google' ending the diagnostic odyssey in lysosomal storage disorders: parents using internet search engines as an efficient diagnostic strategy in rare diseases.

Science.gov (United States)

Bouwman, Machtelt G; Teunissen, Quirine G A; Wijburg, Frits A; Linthorst, Gabor E

2010-08-01

The expansion of the internet has resulted in widespread availability of medical information for both patients and physicians. People increasingly spend time on the internet searching for an explanation, diagnosis or treatment for their symptoms. Regarding rare diseases, the use of the internet may be an important tool in the diagnostic process. The authors present two cases in which concerned parents made a correct diagnosis of a lysosomal storage disorder in their child by searching the internet after a long doctor's delay. These cases illustrate the utility of publicly available internet search engines in diagnosing rare disorders and in addition illustrate the lengthy diagnostic odyssey which is common in these disorders.

A very rare case of bloody tears with enigmatic epistaxis and haematuria.

Science.gov (United States)

Ho, Jason Z S; de Silva, Julian; Olver, Jane

2011-03-01

Bloody tears are a rare symptom that can be caused by local or systemic pathology. We describe a very rare case of bloody tears that resulted from hyperthyroidism. A 15-year-old female patient presented with a 6-month history of bloody tears and epistaxis. Examination excluded local ocular and nasal pathology, including neoplasm and coagulopathy. Systemic investigations identified elevated thyroid function and following treatment her symptoms resolved. We discuss the mechanism by which hyperthyroidism may induce haemostatic dysfunction. We present the first case of bloody tears secondary to thyroid dysfunction.

Rare case of pancreatic cancer with leptomeningeal carcinomatosis

Science.gov (United States)

Yoo, In Kyung; Lee, Hong Sik; Kim, Chang Duk; Chun, Hoon Jai; Jeen, Yoon Tae; Keum, Bora; Kim, Eun Sun; Choi, Hyuk Soon; Lee, Jae Min; Kim, Seung Han; Nam, Seung Joo; Hyun, Jong Jin

2015-01-01

Leptomeningeal carcinomatosis occurs very rarely in patients with pancreatic cancer. Leptomeningeal carcinomatosis is characterized by multifocal seeding of the leptomeninges by malignant cells that originate from a solid tumor. To the best of our knowledge, brain metastasis from pancreatic cancer is extremely rare. Leptomeningeal carcinomatosis is estimated to occur in 3% to 8% of cases of solid tumors. The clinical manifestation usually involves neurological symptoms, including dizziness, headache, vomiting, nausea, and hemiparesis, symptoms similar to those of meningitis or brain tumors. Diagnostic methods for leptomeningeal carcinomatosis include brain magnetic resonance imaging and cerebrospinal fluid examination. Here, we describe a case of leptomeningeal carcinomatosis in which the primary tumor was later determined to be pancreatic cancer. Brain magnetic resonance imaging findings showed mild enhancement of the leptomeninges, and cerebrospinal fluid cytology was negative at first. However, after repeated spinal taps, atypical cells were observed on cerebrospinal fluid analysis and levels of tumor markers such as carbohydrate antigen 19-9 in cerebrospinal fluid were elevated. Abdominal computed tomography, performed to determine the presence of extracerebral tumors, revealed pancreatic cancer. Pancreatic cancer was confirmed histopathologically on examination of an endoscopic ultrasound-guided fine needle aspiration specimen. PMID:25624740

Pediatric gliosarcoma with fibrosarcomatous differentiation: Report of a rare case

Directory of Open Access Journals (Sweden)

Shantha Ravisankar

2012-01-01

Full Text Available Gliosarcoma is a rare variant of glioblastoma with a biphasic pattern showing glial and mesenchymal differentiation. It is seen in adults during their fifth to sixth decades of life and is extremely rare in children. We report a case of primary gliosarcoma with fibrosarcomatous differentiation in an 11-year-old boy presenting with headache and vomiting. Imaging showed a contrast-enhancing isodense space-occupying lesion with areas of calcification in the right temporoparietal cortex. A total excision was done and, on histopathologic examination, a differential diagnostic consideration of gliosarcoma and teratoma with malignant transformation was made. After immunohistochemical analysis, a final diagnosis of gliosarcoma with fibrosarcomatous differentiation was then made. Primary gliosarcoma is a very rare tumor in children with a poor prognosis.

Left Sided Amyand's Hernia, A Rare Occurance: A Case Report.

Science.gov (United States)

Ravishankaran, Praveen; Mohan, G; Srinivasan, A; Ravindran, G; Ramalingam, A

2013-06-01

This is a case report about a 35 year old man admitted with complains of obstructed left sided inguinal hernia. On exploration of the left inguinal canal to our surprise a normal appendix was found in addition to a gangrenous omentum. Resection of the gangrenous omentum was done. Appendectomy was done. This case is reported for its rare occurance as only three such cases of left sided amyand's hernia has been reported so far in literature[4-6].

Neurenteric cyst - a case report of this rare disorder

NARCIS (Netherlands)

Schurink, M.; van Herwaarden-Lindeboom, M. Y. A.; Coppes, M. H.; Veldhuizen, A. G.; Koetse, H. A.; de Langen, Z. J.

Neurenteric cysts are rare congenital lesions that are believed to be the result of the split notochord syndrome. We report the clinical case of a 5-year-old boy presenting with vague gastrointestinal symptoms and fatigue, who had undergone resection of a small intestine duplication cyst as a

Chondroma of Falx: Case Report of a Rare Condition

Directory of Open Access Journals (Sweden)

Shahryar Shahriarian

2012-03-01

Full Text Available Chondroma is a benign tumor which mostly occurs in extremities but also sometimes in brain. Most intracranial chondromas arise from skull base, but chondroma of falx origin is a rare circumstance. Indeed, the intracranial chondromas rise from falx is mostly in relation with syndromic disorders such as Mafuccis syndrome or Olliers syndrome. Here, we reported a rare case of falxian intracranial chondroma in a young man who has normal physical examination and no signs of any syndromic disorder. The goal of this paper was to raise awareness about chondromas and suggest that chondroma be ruled out in any patient with masses arising from falx.

Ankyloglossia with cleft lip: A rare case report

Science.gov (United States)

Jangid, Kritika; Alexander, Aurelian Jovita; Jayakumar, Nadathur Doraiswamy; Varghese, Sheeja; Ramani, Pratibha

2015-01-01

Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman syndrome or Simpson-Golabi-Behmel syndrome. This article presents a rare case of ankyloglossia associated with cleft lip treated with diode laser in a 12-year-old Indian boy who had undergone surgical correction of associated cleft lip soon after birth. Correction of ankyloglossia at a young age would lead to enhanced phonetics, improved oral hygiene, and overall personality development. PMID:26941523

Ankyloglossia with cleft lip: A rare case report

Directory of Open Access Journals (Sweden)

Kritika Jangid

2015-01-01

Full Text Available Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman syndrome or Simpson-Golabi-Behmel syndrome. This article presents a rare case of ankyloglossia associated with cleft lip treated with diode laser in a 12-year-old Indian boy who had undergone surgical correction of associated cleft lip soon after birth. Correction of ankyloglossia at a young age would lead to enhanced phonetics, improved oral hygiene, and overall personality development.

Joubert Syndrome: Imaging Features and Illustration of a Case

International Nuclear Information System (INIS)

Arora, Richa

2014-01-01

Joubert Syndrome (JS) is a rare autosomal recessive disorder characterised clinically by neonatal breathing dysregulation, developmental delay, intellectual disability, hypotonia, ataxia, nystagmus. We present another case of this uncommon syndrome in a 12 years old patient presenting with classical complaints of developmental delay, intellectual impairment, weakness in both lower limbs, ataxia and abnormal facies and diagnosed on Computed Tomography. Joubert Syndrome should be ruled out in all patients presenting with hypotonia, ataxia, nystagmus, breathing abnormalities and developmental delay. Its neuroimaging hallmarks include molar tooth sign and batwing shaped fourth ventricle. As JS is associated with multiorgan involvement, these patients should enter a diagnostic protocol to assess systemic abnormalities. Extreme caution should be taken while administering drugs in these patients as they are prone to respiratory depression

A Rare Case of Malignant Glomus Tumor of the Esophagus

Directory of Open Access Journals (Sweden)

Gurvinder Singh Bali

2013-01-01

Full Text Available Glomus tumors are rare neoplasms that usually occur on the hands in a subungual location, or sometimes in palms, wrists or soles of the feet. They are described as purple/pink tiny painful lesions with a triad of pain, local point tenderness, and cold hypersensitivity. They are almost always benign, but rare malignant variants have been reported. They have also been reported to be present at unusual locations, like the lung, stomach, or liver. Gastrointestinal glomus tumors are extremely rare tumors and very few cases have been reported in the literature. Most that have been reported were usually benign in nature. A rare esophageal glomangioma, mimicking a papilloma, was reported in 2006. We report a case of glomangiosarcoma (malignant glomus tumor in a 49-year-old female, who presented with symptoms of dysphagia including some spasm and hoarseness and subjective unintentional weight loss. On endoscopic exam, she was found to have a distal esophageal mass with malignant features. Radiologically, the mass had a size of about 8 cm on the CT scan without evidence of metastases. Pathology and immunostaining of the biopsy showed features resembling a malignant glomus tumor. She underwent an endoscopic and laparoscopic staging of the tumor along with ultrasound. Based on the laparoscopic findings, which were consistent with the preoperative diagnosis, she was scheduled for an esophagectomy. Histopathology and immunophenotypic features of the excised mass were consistent with a diagnosis of malignant glomus tumor.

Oral malignant melanoma: a rare case with unusual clinical ...

African Journals Online (AJOL)

Primary Oral malignant melanoma is a rare tumor with an indigent prognosis. This is a case report of 47-year-old Sudanese female diagnosed as Oral malignant melanoma of the mandible with an unusual pattern of growth and clinical presentation. Furthermore, a possibility of intraosseous origin is suggested. Pan African ...

Primary intraspinal extradural primitive neuroectodermal tumor: A rare case.

Science.gov (United States)

Rege, Shrikant V; Tadghare, Jitendra; Patil, Harshad; Narayan, Sharadendu

2016-01-01

Primitive neuroectodermal tumors (PNETs) are aggressive childhood malignancies and are difficult to treat. Primary intraspinal PNETs are rare. These patients have poor prognosis with short survival time even after surgery and chemoradiation. As there are no standard guidelines exist for the management of these tumors, a multidisciplinary approach has been employed with varying success. According to the review of literature, only few cases of primary intraspinal extradural PNETs have been reported. Herein, author has described a case of intraspinal, extradural PNET.

[Rare side effects in management of hyperthyroidism. Case report].

Science.gov (United States)

Sohár, Gábor; Kovács, Mónika; Györkös, Andrea; Gasztonyi, Beáta

2016-05-29

The authors present the case history of a patient suffering from hyperthyroidism. The diagnostic procedures revealed the presence of propylthiouracyl induced vasculitis with renal involvement, that recovered completely after the withdrawal of propylthiouracyl and corticosteroid treatment. Thereafter, the patient was treated with thiamasol, that caused agranulocytosis with fever. After transient litium carbonate therapy a succesful thyreoidectomy was performed. Cumulative serious side effects of antithyroid drugs are rare. This case highlights some of the challenges and complications encountered in the management of hyperthyroidism.

Urachal adenocarcinoma: a rare case report

Directory of Open Access Journals (Sweden)

Bo Bao

2017-03-01

Full Text Available Urachal carcinoma is a rare and aggressive form of bladder cancer involving the urachus, a fibrous remnant of the allantois that extends from the bladder to the umbilicus. We report this case of a 49-year-old women with primary urachal adenocarcinoma treated with partial cystectomy who relapsed 5 years after surgery with lung metastases. This patient with unremarkable medical history presented with abdominal discomfort and a palpable pelvic mass. Follow-up imaging reveals a large mass on the dome of the bladder extending from the urachus. Subsequent ultrasound-guided biopsy result was suggestive of an urachal mucinous adenocarcinoma. The patient was treated surgically with a partial cystectomy.

Cystic fibroadenoma: report of a rare case with review of literature.

Science.gov (United States)

Bhat, Amoolya; Vijaya, C; Gowda, V S Shankare

2015-01-01

Fibroadenomas with a predominant cystic change are called cystic fibroadenomas. These are extremely rare forms of fibroadenomas and only one case has been reported so far. They are classified under the category of complex fibroadenomas. Complex fibroadenomas are a rare variant of fibroadenomas occurring in elderly females. They are characterized by presence of one of the complex features along with the usual patterns of fibroadenoma such as cysts more than 3 mm, papillary apocrine metaplasia, or sclerosing adenosis. Patients with these lesions have higher chances of developing carcinoma of breast. We present a case of 35 years old lady with a freely mobile mass in the left breast diagnosed as cystic fibroadenoma after thorough histopathological examination of the lesion.

Brown′s syndrome with ocular albinism: Case report of a rare presentation and literature review

Directory of Open Access Journals (Sweden)

Soumya Nambiar

2015-01-01

Full Text Available We report a rare case of Brown′s syndrome associated with ocular albinism in a 10-year-old boy. This report highlights the importance of further analysis of cases of Brown′s syndrome and throws some light on the etiology and association of this rare condition.

A case report on esophageal tuberculosis – A rare entity

Directory of Open Access Journals (Sweden)

Vatsal Khanna

2017-01-01

Full Text Available This is a case report of a rare form of tuberculosis in a patient presenting with dysphagia. Patient was subjected to upper gastrointestinal endoscopy, which revealed an ulcerative growth in the distal esophagus. Histopathology revealed esophageal tuberculosis. Patient was managed conservatively with Anti-Tuberculosis Treatment (ATT. Follow up endoscopy after two months revealed resolution of the growth and patient was symptomatically better. In spite of the rare nature of the disease, it can be managed effectively with ATT to avoid complications (fistula, stricture, and esophageal perforation, which might warrant surgery.

Gorlin-Goltz syndrome: A rare case report.

Science.gov (United States)

Pol, Chetan A; Ghige, Suvarna K; Kalaskar, Ritesh R; Gosavi, Suchitra R

2013-10-01

Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder characterized by numerous basal cell carcinomas, odontogenic keratocysts (OKCs) and musculoskeletal malformations. A rare case of this syndrome observed in a 13-year-old male patient is presented in which multiple OKCs were causing disfigurement of the lower jaw as well as displacement and malocclusion of the teeth. Early diagnosis and treatment of this syndrome is important to reduce the severity of complications including cutaneous and cerebral malignancy and oromaxillofacial deformation and destruction due to jaw cysts.

A Rare Case Presentation of a Perforated Giant Sigmoid Diverticulum

Directory of Open Access Journals (Sweden)

Jennifer C. Kam

2013-01-01

Full Text Available Giant sigmoid diverticulum (GSD is a rare complication of diverticulosis. These lesions arise from herniations of the mucosa through the muscle wall which progressively enlarge with colonic gas to become large air-filled cysts evident on plain X-ray and CT scans. We present a rare case of a 72-year-old female presenting with abdominal distention, abdominal tenderness, and fever who developed a type 1 giant sigmoid diverticulum (pseudodiverticulum that subsequently formed an intra-abdominal abscess and an accompanying type 2 diverticulum as well. The patient was treated with surgical resection of the diverticulum with a primary anastomosis and abscess drainage. The patient’s postoperative course was uneventful. This case helps to support the need for the consideration of GSD in patients aged 60 and older with a history of diverticulosis and presenting with abdominal discomfort and distension.

A Rare Case: Struma Ovarii in a 14-Year-Old Girl.

Science.gov (United States)

Iltar, Elif; Ureyen, Isin; Toptas, Tayfun; Savas, Melike; Çekiç, Sema; Uysal, Aysel

2018-02-01

Ovarian tumors presented with ovarian mass in childhood and adolescence are uncommon but an important part of gynecological cases. Struma ovarii is one of the rare cystic benign ovarian tumors that is observed predominantly in women who are between the ages of 40 and 60 years old. It is extremely rare in adolescents. Herein, we present a 14-year-old adolescent girl with struma ovarii who presented to the emergency room with abdominal pain.

A Rare Case Report of Bilateral Complex Macrocystic Adrenal Hemorrhage Mimicking Fetal Neuroblastoma

OpenAIRE

Sindhwani, Geetika; Patel, Viral; Jain, Abhinav

2018-01-01

Fetal and neonatal adrenal glands are large vascular organs, which make them vulnerable to frequent bleeding. Although neonatal adrenal hemorrhage is commonly reported, it is rarely diagnosed on antenatal sonography. We present a rare case of prenatally diagnosed bilateral adrenal hemorrhage, which mimicked antenatal neuroblastoma.

Rare case of acute dengue encephalitis with correlated MRI findings

International Nuclear Information System (INIS)

Mathew, Rishi Philip; Basti, Ram Shenoy; Hegde, Pavan; Devdas, Jaidev M.; Khan, Habeeb Ullah; Bukelo, Mario Joseph

2014-01-01

Dengue encephalitis is extremely rare, with most patients showing no significant abnormality on neuroimaging (CT/MRI). We report one of the very few documented cases of dengue encephalitis, with abnormal signal intensities on all major sequences on brain MRI.

Primary Small Cell Neuroendocrine Carcinoma of Vagina: A Rare Case Report

Directory of Open Access Journals (Sweden)

Jignasa N. Bhalodia

2011-01-01

Full Text Available Primary small cell neuroendocrine carcinoma of vagina is an extremely rare disease. There have been only 26 previously reported cases in literature. Here, we report a case of primary small cell neuroendocrine carcinoma of vagina. Immunohistochemistry (IHC showed tumor cells positive for synaptophysin, chromogranin, and neuron-specific enolase (NSE.

The Missing Tooth: Case Illustrations of a Child's Assembled, Out-of-School Authorship

Science.gov (United States)

Winters, Kari-Lynn

2012-01-01

Case illustrations of a six-year-old boy's adventures with a missing tooth are used in this paper to re-define a broader notion of authorship. Drawing on theories of social semiotics, New Literacy Studies (NLS), and critical positioning, this notion of authorship not only interweaves the boy's preferred modes of meaning-making and communication,…

Maxillo-Mandibular Cemento-ossifying Fibroma: A Rare Case Report.

Science.gov (United States)

Peravali, Ranjit Kumar; Bhat, H Hari Kishore; Reddy, Sreenatha

2015-03-01

Cemento-ossifying fibroma (COF) is a benign fibro osseous lesion of the jaws which has been described as a demarcated or rarely encapsulated neoplasm consisting of fibrous tissue and varying amounts of mineralized material resembling bone and/or cementum (Dinkar et al. in IJDA 2(4):45-47, 2010). Majority of lesions occur in the mandible and only few cases of COFs of the maxillary sinus and bilateral COFs of the mandible have been reported in literature (Dinkar et al. in IJDA 2(4):45-47, 2010; Tamiolakis et al. in Acta Stomatol Croat 39(3):319-321, 2005; Hamner et al. in Oral Surg Oral Med Oral Pathol 26(4):579-587, 1968; Gunaseelan et al. in Oral Med Oral Pathol Oral Radiol Endod 104:e21-e25, 2007). These lesions have a very low recurrence rate (Ertug et al. in Quintessence Int 35(10):808-810, 2004) and are generally treated by enucleation. In this paper we present a rare case of COF occurring in both the maxilla and mandible of the same patient. Only one such case (Takeda and Fujioka in Int J Oral Maxillofac Surg 16(3):368-371, 1987) has been reported in literature so far.

Transverse Vaginal Septum With Secondary Infertility: A Rare Case.

Science.gov (United States)

Rahman, Hafeez; Trehan, Nikita; Singh, Shuchita; Goyal, Meenakshi

2016-01-01

To demonstrate the technique of laparoscopic vaginal reconstruction in a rare case of mid-vaginal septum with secondary infertility. A step-by-step explanation of the technique using videos and pictures (Canadian Task Force classification IV). Transverse vaginal septum is a rare condition, with an incidence of only 1 in 30,000 women. It is usually a congenital mullerian fusion defect; few cases of acquired septum have been reported. Roughly 40% of cases occur in the mid-vagina. Transverse vaginal septum typically presents with primary amenorrhea and hematocolpos. The goal of surgery is to create a patent vagina with restoration of fertility. The laparoscopic approach has proven superiority over the open technique. Laproscopic vaginal reconstruction was performed in a 24 year female with transverse vaginal septum. Dissection was done laproscopically up to mid vagina. Incision was given on vagina excising the septum followed by reconstruction. The laparoscopic approach to vaginal reconstruction avoids the abdominal incision, with its associated pain and possible adhesion formation. It also provides a better view for dissection. In this patient, a patent vagina was created in a single operation, with no postoperative dyspareunia, and fertility was restored. Copyright © 2016 AAGL. Published by Elsevier Inc. All rights reserved.

Bilateral double-headed condyles: A rare case report

Directory of Open Access Journals (Sweden)

Swati Phore

2018-01-01

Full Text Available Bifid mandibular condyle is characterized by the duplicity of the head of the mandibular condyle, so the name double-headed condyle. It is usually diagnosed on routine radiographic examination and is described as a rare entity. Usually, bifid condyle is an incidental finding and its etiology is controversial, with no predilection for sex or ethnic background. Herein, we report a case of bilateral bifid condyles.

Ultrasound, CT and MRI Appearances of a Rare Symptomatic Laryngeal Chondrometaplasia: A Case Report

International Nuclear Information System (INIS)

Lee, Ryan Ka Lok; Hok Yuen, Edmond Yuen; Abdullah, Victor James; Ping Lee, Yolanda Yim; Ahuja, Anil Tejbhan

2015-01-01

Symptomatic laryngeal chondrometaplasia is rare. To the best of our knowledge, there are only few case reports on laryngeal chondrometaplasia. The imaging appearance of this uncommon disease is even more rarely described. There are only two case reports describing its appearances in computed tomography (CT) and magnetic resonance imaging (MRI). Ultrasound (US) features have not been reported so far. This case report is to show the US, CT and MRI features of this disease entity to stress the role of imaging in this disease

Rare presentation of pancreatic schwannoma: a case report

Directory of Open Access Journals (Sweden)

Tofigh Arash

2008-08-01

Full Text Available Abstract Introduction Schwannoma is a rare tumor among pancreatic neoplasms. Schwannomas vary in size, and most of them are cystic, mimicking pancreatic cystic lesions. Generally, a definitive diagnosis is made at the time of histological analysis. The mainstay treatment is surgical resection. Case presentation We report an unusual presentation of pancreatic schwannoma with abdominal pain and several episodes of cholangitis in a 54-year-old Caucasian (Iranian man. The condition was not diagnosed pre-operatively and Whipple's procedure was performed. Conclusion Pancreatic schwannoma is an important clinical entity to include in the differential diagnosis of pancreatic lesions. Pre-operative diagnosis is difficult but computed tomographic findings may be helpful. The tumor may also have atypical and rare presentations, such as cholangitis and weight loss. For benign tumors, simple enucleation is usually adequate, whereas malignant tumors require standard oncological resection.

Cerebrotendinous xanthomatosis (a rare lipid storage disorder): a case report

OpenAIRE

Razi, Syed Mohd; Gupta, Abhinav Kumar; Gupta, Deepak Chand; Gutch, Manish; Gupta, Keshav Kumar; Usman, Syeda Iqra

2016-01-01

Background Cerebrotendinous xanthomatosis is a very rare autosomal recessive lipid storage disorder affecting bile acid biosynthesis. It is manifested by subtle neurological and non-neurological symptoms due to abnormal tissue lipid deposition. Diagnosis is usually delayed but early diagnosis and replacement therapy can prevent devastating neurological sequelae. Case presentation We present a case of a 25-year-old Asian Indian woman who presented with gait difficulty, fusiform swellings of bi...

A Rare Case of Michelin Tire Baby Syndrome in a Newborn.

Science.gov (United States)

Ramphul, Kamleshun; Mejias, Stephanie G; Ramphul-Sicharam, Yogeshwaree

2018-02-24

Kunze-Riehm syndrome also known as Michelin tire baby syndrome (MTBS) is a rare genetic condition with a characteristic generalized folding of excess skin. The diagnosis is usually made based on clinical symptoms. There are approximately only 30 cases reported in the literature and some cases were associated with non-cutaneous anomalies as well. Herein, we report a case of MTBS in a five-day-old male of Iraqi origin.

Progeria in siblings: A rare case report

Directory of Open Access Journals (Sweden)

R Sowmiya

2011-01-01

Full Text Available Progeria, also known as Hutchinson-Gilford syndrome, is an extremely rare, severe genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. It is an autosomal dominant disorder. It is not seen in siblings of affected children although there are very few case reports of progeria affecting more than one child in a family. Here we are presenting two siblings, a 14-year-old male and a 13-year-old female with features of progeria, suggesting a possible autosomal recessive inheritance.

Gorlin-Goltz syndrome: A rare case report

Directory of Open Access Journals (Sweden)

Chetan A Pol

2013-01-01

Full Text Available Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder characterized by numerous basal cell carcinomas, odontogenic keratocysts (OKCs and musculoskeletal malformations. A rare case of this syndrome observed in a 13-year-old male patient is presented in which multiple OKCs were causing disfigurement of the lower jaw as well as displacement and malocclusion of the teeth. Early diagnosis and treatment of this syndrome is important to reduce the severity of complications including cutaneous and cerebral malignancy and oromaxillofacial deformation and destruction due to jaw cysts.

Papilliferous Keratoameloblastoma: An Extremely Rare Case Report

Directory of Open Access Journals (Sweden)

Neeta Mohanty

2013-01-01

Full Text Available Odontogenic tumors develop in the jaw bones from the odontogenic tissue-oral epithelium in tooth germ, enamel organ, dental papilla, reduced enamel epithelium, remnants of Hertwig’s root sheath or dental lamina, and so forth. Hence, a bewildering variety of tumors are encountered in the maxilla and mandible. Ameloblastoma is the second most common odontogenic neoplasm after odontomes, and it has numerous clinical and histologic variants. We report a very rare histologic variant: the papilliferous keratoameloblastoma which is the fifth reported case in the English literature.

[A rare case of diencephalic cachexia in an adult female with cranio-pharyngioma].

Science.gov (United States)

Klochkova, I S; Astaf'eva, L I; Konovalov, A N; Kadashev, B A; Kalinin, P L; Sharipov, O I; Kutin, M A; Sidneva, Yu G; Shishkina, L V; Pronin, I N

Diencephalic cachexia (DС) is progressive weight loss despite a normal caloric intake and a satisfactory state of health, which is caused by hypothalamic lesions. This is a rare (about 100 cases were reported) and potentially fatal disorder of unknown pathogenesis. At present, there is no effective pharmacological therapy for the disorder. Cachexia may regress only if the tumor reduces in size, therefore the timely diagnosis and treatment are of vital importance for the patient. DС is typical of early childhood, and only a few cases have been reported in adults. We present a rare case of DС in a 24-year-old female with papillary craniopharyngioma.

Illustrating, Quantifying, and Correcting for Bias in Post-hoc Analysis of Gene-Based Rare Variant Tests of Association

Directory of Open Access Journals (Sweden)

Kelsey E. Grinde

2017-09-01

Full Text Available To date, gene-based rare variant testing approaches have focused on aggregating information across sets of variants to maximize statistical power in identifying genes showing significant association with diseases. Beyond identifying genes that are associated with diseases, the identification of causal variant(s in those genes and estimation of their effect is crucial for planning replication studies and characterizing the genetic architecture of the locus. However, we illustrate that straightforward single-marker association statistics can suffer from substantial bias introduced by conditioning on gene-based test significance, due to the phenomenon often referred to as “winner's curse.” We illustrate the ramifications of this bias on variant effect size estimation and variant prioritization/ranking approaches, outline parameters of genetic architecture that affect this bias, and propose a bootstrap resampling method to correct for this bias. We find that our correction method significantly reduces the bias due to winner's curse (average two-fold decrease in bias, p < 2.2 × 10−6 and, consequently, substantially improves mean squared error and variant prioritization/ranking. The method is particularly helpful in adjustment for winner's curse effects when the initial gene-based test has low power and for relatively more common, non-causal variants. Adjustment for winner's curse is recommended for all post-hoc estimation and ranking of variants after a gene-based test. Further work is necessary to continue seeking ways to reduce bias and improve inference in post-hoc analysis of gene-based tests under a wide variety of genetic architectures.

A Rare Case Report of Immobility-Induced Hypercalcemia in an Infant.

Science.gov (United States)

Vyas, Neha; Kaminski, Beth; MacLeish, Sarah

2016-04-01

Immobility-induced hypercalcemia is a rare cause of hypercalcemia in children, and to our knowledge it has never been reported in an infant. Infants and children are in a state of high bone turnover. Therefore, they are prone to the imbalance of osteoblastic and osteoclastic activity that occurs with prolonged immobilization, leading to hypercalcemia. Here we present the case of an infant with hypercalcemia who presented with fatigue, irritability, and failure to thrive after prolonged immobilization. Therapeutic interventions were conservative and included hydration and increased mobility leading to complete resolution. This case highlights the importance of including this rare entity in a differential diagnosis of hypercalcemia as well as screening postsurgical patients with prolonged immobility for hypercalcemia. Copyright © 2016 by the American Academy of Pediatrics.

A rare case of bleeding disorder: Glanzmann's thrombasthenia.

Science.gov (United States)

Swathi, Jami; Gowrishankar, A; Jayakumar, S A; Jain, Karun

2017-01-01

Glanzmann's thrombasthenia (GT) is a rare bleeding disorder, which is characterized by a lack of platelet aggregation. It is characterized by qualitative or quantitative abnormalities of the platelet membrane glycoprotein IIb/IIIa. Physiologically, this platelet receptor normally binds several adhesive plasma proteins, and this facilitates attachment and aggregation of platelets to ensure thrombus formation at sites of vascular injury. The lack of resultant platelet aggregation in GT leads to mucocutaneous bleeding whose manifestation may be clinically variable, ranging from easy bruising to severe and potentially life-threatening hemorrhages. To highlight this rare but potentially life-threating disorder, GT. We report a case of GT that was first detected because of the multiple episodes of gum bleeding. The patient was an 18-year-old girl who presented with a history of repeated episodes of gum bleeding since childhood. Till the first visit to our hospital, she had not been diagnosed with GT despite a history of bleeding tendency, notably purpura in areas of easy bruising, gum bleeding, and prolonged bleeding time after abrasions and insect stings. GT was diagnosed on the basis of prolonged bleeding time, lack of platelet aggregation with adenosine di phosphate, epinephrine and collagen. GT should always be considered as differential diagnosis while evaluating any case of bleeding disorder.

Adult-onset cystic hygroma: A case report of rare entity.

Science.gov (United States)

Bahl, Sumit; Shah, Vandana; Anchlia, Sonal; Vyas, Siddharth

2016-01-01

Cystic hygroma is a benign congenital malformation of the lymphatic system that occurs in infant or children younger than 2 years of age. Although cystic hygroma is well recognized in pediatric practice, it seldom presents de novo in adulthood. These are commonly present in head and neck but can be present anywhere. Cystic hygroma is very rare in adults, but it should be considered in the differential diagnosis of adult neck swellings. Patients presenting with a painless, soft, fluctuant, and enlarging neck mass should have a careful history and physical examination along with radiological imaging to assist with diagnosis. Surgical intervention is the treatment of choice for this rare condition. Here, we are reporting a case of cystic hygroma in a 32-year-old male patient in the neck region. The objectives of this case report are to discuss the clinical presentation, diagnosis, histopathological findings and management of this malformation.

A rare case of short stature: Say Meyer syndrome

OpenAIRE

Karthik, T. S.; Prasad, N. Rajendra; Rani, P. Radha; Maheshwari, Rushikesh; Reddy, P. Amaresh; Chakradhar, B. V. S.; Menon, Bindu

2013-01-01

Introduction: Say Meyer syndrome is rare X linked condition characterized by developmental delay, short stature and metopic suture synostosis. We are reporting a case of Say Meyer syndrome presented to our hospital for short stature and developmental delay at age 3½ years. Case Report: A 3½-year-old boy presented to our hospital for decreased growth velocity from the age of 1 year. History revealed the boy had a birth weight of 2.3 kg, had an episode of seizures in the neonatal period. He was...

Unusually large erupted complex odontoma: A rare case report

Energy Technology Data Exchange (ETDEWEB)

Bagewadi, Shivanand B.; Kukreja, Rahul; Suma, Gundareddy N.; Yadav, Bhawn; Sharma, Havi [Dept. of Oral Medicine and Radiology, ITS Centre for Dental Studies and Research, Murad Nagar (India)

2015-03-15

Odontomas are nonaggressive, hamartomatous developmental malformations composed of mature tooth substances and may be compound or complex depending on the extent of morphodifferentiation or on their resemblance to normal teeth. Among them, complex odontomas are relatively rare tumors. They are usually asymptomatic in nature. Occasionally, these tumors become large, causing bone expansion followed by facial asymmetry. Odontoma eruptions are uncommon, and thus far, very few cases of erupted complex odontomas have been reported in the literature. Here, we report the case of an unusually large, painless, complex odontoma located in the right posterior mandible.

Sialadenoma papilliferum: A rare case report and review of literature

Directory of Open Access Journals (Sweden)

S Sunil

2017-01-01

Full Text Available Sialadenoma papilliferum (SP classified under the ductal papillomas by the WHO is a rare benign tumor of minor salivary glands. It is a rare lesion of salivary glands predominantly affecting the minor glands. It has characteristic exophytic and endophytic clinical growth pattern. Histopathologically, it is characterized by papillary projections supported by fibrovascular connective tissue core and infiltrated with mixed inflammatory cells. The ductal lining epithelium of double-layered cells of luminal layer of tall columnar cells and a basilar layer of small cuboidal cells shows additional papillary projections into the lumen. We report a case of SP of mid palate.

Sirenomelia: A Rare Case of Foetal Congenital Anomaly

OpenAIRE

Dharmraj, Meena; Gaur, Sumitra

2012-01-01

Sirenomelia, alternatively known as ?mermaid syndrome? is a very rare congenital deformity in which the legs are fused together, giving them the appearance of the tail of a mermaid?. Other birth defects are always associated with sirenomelia, most commonly abnormalities of the kidneys, large intestines, and genitalia. The present case is a one of sirenomelia associated with an absent right kidney, mild left hydronephrosis, single umbilical artery, and severe oligohydramnios. We discuss the fi...

The detection of clusters in rare diseases

Energy Technology Data Exchange (ETDEWEB)

Besag, J. (Washington Univ., Seattle, WA (USA) Newcastle upon Tyne Univ. (UK)); Newell, J. (Newcastle upon Tyne Univ. (UK))

1991-01-01

Tests for clustering of rare diseases investigate whether an observed pattern of cases in one or more geographical regions could reasonably have arisen by chance alone, bearing in mind the variation in background population density. In contrast, tests for the detection of clusters are concerned with screening a large region for evidence of individual 'hot spots' of disease but without any preconception about their likely locations; the results of such tests may form the basis for subsequent small area investigations, statistical or non-statistical, but will rarely be an end in themselves. The main intention of the paper is to describe and illustrate a new technique for the identification of small clusters of disease. A secondary purpose is to discuss some common pitfalls in the application of tests of clustering to epidemiological data. (author).

A rare case of benign omentum teratoma

Directory of Open Access Journals (Sweden)

Sforza Marcos

2012-01-01

Full Text Available Introduction. Mature teratomas (benign cystic teratomas or dermoid cysts are among the most common ovarian tumours; however, teratomas of the omentum and mesentery are extremely rare. Teratoma in the intraperitoneal cavity is uncommon and atypical, and it is even more uncommon in adulthood. Case Outline. An 82-year-old female was admitted to our department with clinical signs of abdominal tumour. The ultrasound scan and preoperative laboratory tests were done. Explorative laparotomy revealed tumour with torsion on its pedicle at the greater omentum. After removal of the mass and the incision a tooth and hair were found, characteristics of teratoma. Conclusion. The excision was very effective and also definitive treatment for this case. The patient recovered well and was discharged 3 days later. The patient probably carried the tumour all her life asymptomatically until admission.

Detecting rare variants in case-parents association studies.

Directory of Open Access Journals (Sweden)

Kuang-Fu Cheng

Full Text Available Despite the success of genome-wide association studies (GWASs in detecting common variants (minor allele frequency ≥0.05 many suggested that rare variants also contribute to the genetic architecture of diseases. Recently, researchers demonstrated that rare variants can show a strong stratification which may not be corrected by using existing methods. In this paper, we focus on a case-parents study and consider methods for testing group-wise association between multiple rare (and common variants in a gene region and a disease. All tests depend on the numbers of transmitted mutant alleles from parents to their diseased children across variants and hence they are robust to the effect of population stratification. We use extensive simulation studies to compare the performance of four competing tests: the largest single-variant transmission disequilibrium test (TDT, multivariable test, combined TDT, and a likelihood ratio test based on a random-effects model. We find that the likelihood ratio test is most powerful in a wide range of settings and there is no negative impact to its power performance when common variants are also included in the analysis. If deleterious and protective variants are simultaneously analyzed, the likelihood ratio test was generally insensitive to the effect directionality, unless the effects are extremely inconsistent in one direction.

Ocular side effect of tinidazole: A rare case report

Directory of Open Access Journals (Sweden)

Hina Kauser

2014-01-01

Full Text Available Ocular side effects in the form of punctate epithelial erosions with the use of tinidazole - a 5-nitroimidazole group of drugs is very rare. A 32-year-old male was prescribed tablet tinidazole for the treatment of amoebiasis but developed adverse effects in the form of blisters on both upper and lower lips with itching and burning sensation, itching and burning on penile and anal area associated with punctate epithelial erosions of cornea of both the eyes. All these are rare manifestations but punctate epithelial erosions of cornea has never been reported in the literature so far. Punctate epithelial erosions of cornea have not previously been reported and should be added to the list of complications of tinidazole. Hence, this case is being reported .

Cerebral Venous Thrombosis and Venous Infarction: Case Report of a Rare Initial Presentation of Smoker’s Polycythemia

Directory of Open Access Journals (Sweden)

Mihir Raval

2010-11-01

Full Text Available Introduction: Cerebral venous thrombosis is a rare initial presentation of polycythemia. If diagnosed early, treatment can reduce mortality and morbidity significantly. Often it may present with headache as the only complaint, and thus the diagnosis is likely to be missed. Case Presentation: A medically stable 31-year-old male, a chronic smoker with a ∼17 pack-year history of smoking, was admitted to the emergency room with a 2-week history of gradually worsening, severe, throbbing headache in the occipital region sensitive to light. Initial neurological examination was positive only for some involuntary motor tics of the left leg. Initial laboratory workup showed hemoglobin of 20 g/dl and hematocrit of 56.5%. The carboxyhemoglobin level was normal, but the oxygen dissociation curve was shifted to the left. Further evaluation by MRI and MRA of the brain suggested extensive and complete thrombosis of the superior sagittal sinus, right transverse sinus and right sigmoid sinus with a small venous infarct in the right parafrontal region. Given that the patient first presented with a thrombotic event, workup for primary polycythemia and hypercoagulable disorders was carried out, including JAK2 mutation evaluation, which was negative. This left us with smoking as the only risk factor and possible cause for secondary polycythemia. He improved significantly with phlebotomy and anticoagulation treatment. Conclusion: This case illustrates a rare but severe complication of secondary polycythemia stressing the importance of being aware of the risk of developing cerebral thrombosis in patients with chronic smoking exposure.

A rare cause of chronic mesenteric ischemia from fibromuscular dysplasia: a case report

Directory of Open Access Journals (Sweden)

Senadhi Viplove

2010-11-01

, which revealed diffuse colonic lymphoid reactive hyperplasia. A small bowel series was negative for any inflammation. An indium scan, pan-computed tomography scan and transvaginal ultrasound were also negative. Magnetic resonance angiography of her abdomen revealed proximal superior mesenteric artery stenosis, which was confirmed by computed tomography angiogram findings of severe proximal and distal superior mesenteric artery stenosis, consistent with the appearance of fibromuscular dysplasia on angiography in the absence of vasculitis or atherosclerotic disease. The patient's superior mesenteric artery stenosis was subsequently angioplastied suboptimally and had to be stented with an Angioplus stent. One month after she was admitted, her abdominal pain and tolerance to oral feeds improved tremendously. Conclusion Fibromuscular dysplasia most commonly presents with renal artery stenosis, which rarely causes abdominal pain. This case illustrates how fibromuscular dysplasia can present as a rare cause of chronic mesenteric ischemia, similar to chronic mesenteric ischemia from atherosclerosis.

An Unusual Presentation of Metanephric Adenofibroma: A Rare Case Report

Directory of Open Access Journals (Sweden)

Kiran Agarwal

2017-10-01

Full Text Available Metanephric adenofibroma is a rare renal neoplasm with only a few case reports in literature. In majority of cases, it is asymptomatic. However, it may present with haematuria, polycythemia or hypertension. Radiologically, it is indistinguishable from other solid renal tumours. Definitive diagnosis can only be made on the basis of histopathology. It is a benign neoplasm and requires only surgical excision with no need for chemotherapy. Involvement of urinary bladder and presentation as bladder mass has never been reported. In this case report, we present a case of metanephric adenofibroma in a two-year-old male child manifesting with haematuria and urinary bladder mass.

A Rare Case: Gastric Cancer; Involving Primery Thoracal Vertebral Metastases

Directory of Open Access Journals (Sweden)

Harun Arslan

2013-06-01

Full Text Available Primery bone metastases rarely occur in gastric cancer. Bone metastases indicate that the prognosis is bad. In that article we present a case that is diagnosed as a gastric cancer with primary bone metasteses that caused pathologic thoracal vertebral fracture seenby computer ised tomography.

Intussusception due to a cecal duplication cyst: a rare cause of acute abdomen. Case report.

Science.gov (United States)

Corroppolo, M; Zampieri, N; Erculiani, E; Cecchetto, M; Camoglio, F S

2007-01-01

Duplications of the alimentary tract are rare congenital anomalies. The ileum is the most common site, whereas rectal, duodenal, gastric and cecal duplications are extremely rare. Duplication cysts of the cecum, in a neonate, are even rarer, with only 19 cases reported in medical literature to date. We report a case of intestinal intussusception due to a cecal duplication cyst.

A Rare Case of Pneumonia Caused by Shewanella putrefaciens

Directory of Open Access Journals (Sweden)

Rajshree Patel

2012-01-01

Full Text Available Shewanella putrefaciens is a gram-negative, nonfermentative, oxidase positive, motile bacillus that produces hydrogen sulphide. It is found widely in the nature especially in marine environments. In some very rare cases Shewanella putrefaciens can be a human pathogen. It can produce a wide variety of clinical syndromes including bacteremia as well as skin and soft tissue infections. However, pneumonia due to S. putrefaciens is rare; there are a total of 4 reported cases in the literature. We present a case of 63-year-old male who was presented to emergency room status after cardiac arrest, fell into sea water face down. On the second day of hospitalization, he was diagnosed to have pneumonia based on the clinical, radiological, and laboratory findings. Empirical antibiotic treatment with vancomycin and piperacillin/tazobactam combination was initiated. Gram-stained smear of endotracheal aspirate yielded gram-negative bacteria, and the isolate grown from endotracheal aspirate culture was identified as S. putrefaciens by Biomerieux API 20 NE technique. On review of the literature and according to culture and sensitivity results, therapy in our patient was changed to cefepime. Patient’s pneumonia improved with treatment with cefepime. We believe that our patient developed pneumonia evidently caused by S. putrefaciens, after near drowning in sea water. The pneumonia resolved after treatment with cefepime.

Intraosseous adenoid cystic carcinoma of maxilla: A rare case report

Directory of Open Access Journals (Sweden)

Prasannasrinivas Suresh Deshpande

2013-01-01

Full Text Available Adenoid cystic carcinoma (ACC accounts for approximately 6-10% of all salivary gland tumors. Palatal minor salivary glands, parotid, and sub-mandibular glands are usually affected. Rarely, these lesions arising intraosseously have been reported. Mandible is commonly involved than maxilla. The present case is a giant ACC involving the right maxilla. A thorough clinical and radiographic evaluation was performed to assess the involvement of surrounding vital structures along with a meticulous metastatic work-up. Computed tomography showed a giant lesion in maxilla encroaching the left nasal fossa, antrum, buccal space, and oral cavity. No metastasis was noted. Histological evaluation from multiple sites showed both cribriform and solid patterns. Radiotherapy was given as patient did not comply for surgery. Though central ACC is extremely rare, especially in maxilla, it should be included in the differentials for lesions in maxilla. A prompt diagnosis with treatment and long-term follow-up is advised in such cases.

A rare case of congenital Y-type urethral duplication

Directory of Open Access Journals (Sweden)

Charu Tiwari

2015-11-01

Full Text Available Duplication of urethra is a rare congenital anomaly. We report a case of Y-type of urethral duplication with the accessory urethra arising from posterior urethra and opening in the perineum. The orthotopic urethra was normal. The accessory urethral tract was cored, transfixed and divided. At 1 year of follow-up, the patient has no urinary complaints

Penile Duplication and Two Anal Openings; Report of a Very Rare Case

OpenAIRE

Bakheet, Mohamed Abdel Al M.; Refaei, Mohammad

2012-01-01

Background Penile duplication (diphallus) is an extremely rare disorder. It is almost always associated with other malformations like double bladder, exstrophy of the cloacae, imperforate anus, duplication of the rectosigmoid and vertebral deformities. Meanwhile anal canal duplication, the most distal and least common duplication of the digestive tube and is a very rare congenital malformation. Case Presentation A 21 days old Egyptian neonate is reported with complete penile duplication and t...

Acute Carbon Monoxide Poisoning Resulting in ST Elevation Myocardial Infarction: A Rare Case Report

Directory of Open Access Journals (Sweden)

Po-Chao Hsu

2010-05-01

Full Text Available Acute carbon monoxide (CO poisoning with cardiac complications is well documented in the literature. However, ST segment elevation is a rare presentation, and most of these cases with ST elevation have revealed non-occlusive or normal coronary arteries. We report a case of CO poisoning complicated with ST elevation myocardial infarction. Emergency coronary angiography revealed total occlusion of the left anterior descending artery and primary percutaneous coronary intervention was performed. This report of a rare case should remind physicians that cardiovascular investigations, including electrocardiography, must be performed in cases with CO poisoning because mortality might increase if reperfusion therapy or appropriate medical treatments are not performed in patients with acute coronary artery occlusion.

A rare case of acute poster ior reversible encephalopathy syndrome involving brainstem in a child

Directory of Open Access Journals (Sweden)

Olfa Chakroun-Walha

2016-11-01

Full Text Available Posterior reversible encephalopathy syndrome (PRES is a rare entity involving brainstem in very rare reported cases. We describe here the case of a boy who presented to the emergency department for headaches and strabismus. Diagnosis of PRES was retained by magnetic resonance imaging. The causes were blood pressure urgency and renal failure. Location of lesions was very rarely reported in literature and neurological troubles were persistent. Emergency physicians should evocate PRES each time there is a clinical context associated with neurological troubles by a normal brain CT scan. Early diagnosis is very important to treat its causes and improve prognosis.

A rare case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA

Directory of Open Access Journals (Sweden)

Meleha Ahmad

2016-12-01

Conclusions and importance: PPRCA is typically bilateral and symmetric, affecting primarily the outer retina and choroid. However, in rare cases, this disease can present unilaterally and/or unifocally, with degeneration extending to the inner retinal layers.

A rare case of vulvar squamous cell carcinoma; case presentation

Directory of Open Access Journals (Sweden)

Cristina N. Cozma

2018-05-01

Full Text Available Objectives. Vulvar cancer is a rare gynecological malignancy, with an incidence of 1.5 per 100 000 women/year. The most common vulvar cancer is developed in squamous cells, the most encountered type of skin cells. Case report. We report a case of a 72-year-old female admitted in the Department of Plastic Surgery of Emergency Clinical Hospital “Prof. Dr. Agrippa Ionescu” with a 5/4.2 cm painful ulcerated tumoral mass located in the vulvar area. The lesion slowly increased in size over the past 12 months. The tumour was surgically removed with oncological safety margins and sent for histopathological evaluation. The histopathological examination revealed an ulcerated squamous carcinoma with lymphovascular and perineural invasion, but with negative margins. Postoperative results were favorable, and no local or general complications were observed. Conclusion. We highlight this case due to its unusual presentation in the clitoral area. Moreover, considering the potential for recurrence we point out the importance of the radical vulvectomy with regional lymphadenectomy and histopathological examination, in order to put a precise diagnosis and ensure the best possible treatment for the patient.

Eye and rare genetic diseases: Case series and literature review ...

African Journals Online (AJOL)

Genetic diseases are generally characterised by a multi visceral pathogenesis. Although orphan, these diseases interest many disciplines due to their clinical expression. Eye is sometimes part of the clinical polymorphism of some rare genetic diseases. Ocular signs are in some cases leading to the diagnosis of these ...

An Integrative Psychotherapy Approach to Foster Community Engagement and Rehabilitation in Schizophrenia: A Case Study Illustration.

Science.gov (United States)

Kukla, Marina; Whitesel, Frankie; Lysaker, Paul H

2016-02-01

This case study illustrates the use of a long-term integrative psychotherapy approach with a middle- aged man with chronic schizophrenia and a mood disorder. The case of "Holst" describes a man with a history of insecure attachment and trauma who later went on to contract a serious chronic illness, precipitating the onset of psychotic symptoms, depression, and chronic suicidal ideation, resulting in multiple hospitalizations. Combining metacognition-oriented therapy with elements of cognitive behavioral therapy and psychiatric rehabilitation, this approach fostered significantly improved community functioning and attainment of personal goals over time. Through the journey of therapy, the patient also developed a more coherent narrative about his life, established a stable sense of self, and became an active agent in the world. This case illustration demonstrates that these three different approaches can be used in a sequential and complementary fashion to foster recovery in the midst of serious physical and mental illness. © 2015 Wiley Periodicals, Inc.

A rare case of retained fourth molar teeth in maxilla and mandible. Case report

Directory of Open Access Journals (Sweden)

Rahnama Mansur

2014-06-01

Full Text Available The study presents a case of the rarely occurring totally retained fourth molar teeth simultaneously in maxilla and mandible. The appearance of supernumerary teeth is a relatively uncommon dental anomaly and it is rare for patients to have impacted fourth molars in two quadrant. The aim of this work is to describe the presence of unilateral (right fourth molars in the maxilla and the mandible in a young female patient aged 24 years. Orthopantomogram revealed impacted lower third molars but also unerupted unilateral (right upper and lower fourth molars. Before orthodontic treatment, the patient was subsequently admitted for removal of third and fourth impacted upper and lower molars under local anesthesia.

Dens evaginatus and dens invaginatus in a double tooth: A rare case report

Directory of Open Access Journals (Sweden)

Gaurav Sharma

2015-01-01

Full Text Available The presence of dens invaginatus (DI and dens evaginatus (DE on same tooth is a rare phenomenon. However, when these dental anomalies occur on a double tooth, it becomes an extremely rare phenomenon. The authors report a rare case of DI and DE on fused permanent maxillary central incisor with supernumerary tooth in a 40-year-old male. The present article also focuses on the differentiating fusion from gemination and also reviews preventive and management strategies for tooth with complex dental anatomy.

Coexistence of morphea and granuloma annulare: a rare case report

Directory of Open Access Journals (Sweden)

Şenay Ağırgöl

2017-11-01

Full Text Available ABSTRACT CONTEXT: Localized scleroderma (morphea is characterized by fibrosis of skin and subcutaneous tissue. Granuloma annulare is a relatively common disease that is characterized by dermal papules and arciform plaques. CASE REPORT: Here, we present the case of a 42-year-old woman who developed granuloma annulare on the dorsum of her feet and abdominal region, and morphea on the anterior side of her lower limbs. We also discuss the etiological and pathogenetic processes that may cause the rare coexistence of these two diseases. CONCLUSION: Only a few cases in the literature have described coexistence of morphea and granuloma annulare.

Report from Mongolia – How much do we know about the incidence of rare cases in less developed countries: a case series

Directory of Open Access Journals (Sweden)

Dünser Martin W

2008-11-01

Full Text Available Abstract Introduction Case reports are important instruments to describe rare disease conditions and give a rough estimation of their global incidence. Even though collected in international databases, most case reports are published by clinicians from industrialized nations and little is known about the incidence of rare cases in less developed countries, which are home to 75% of the world's population. Case presentation We present seven patients who suffered from diseases which are either considered to be rare or have not yet been described before according to international databases, but occurred during a 5-month period in one intensive care unit of a less developed country. During the observation period, patients with a spontaneous infratentorial subdural hematoma (Asian, female, 41 years, general exanthema and acute renal failure after diesel ingestion (Asian, male, 30 years, transient cortical blindness complicating hepatic encephalopathy (Asian, female, 49 years, Fournier gangrene complicating acute necrotizing pancreatitis (Asian, male, 37 years, acute renal failure due to acetic acid intoxication (Asian, male, 42 years, haemolytic uremic syndrome following septic abortion (Asian, female, 45 years, and a metal needle as an unusual cause of chest pain (Asian, male, 41 years were treated. According to the current literature, all seven disease conditions are considered either rare or have so far not yet been reported. Conclusion The global incidence of rare cases may be underestimated by contemporary international databases. Diseases which are currently considered to be rare in industrialized nations may occur at a higher frequency in less developed countries. Reasons may not only be a geographically different burden of certain diseases, limited diagnostic and therapeutic facilities, but also a relevant publication bias.

Cervical Paraganglioma Mimicking Thyroid Nodule: A Rare Clinical Case

Directory of Open Access Journals (Sweden)

Berna İmge Aydoğan

2016-01-01

Full Text Available Objective. Paraganglioma is a rare neuroendocrine tumor. When it is located in the neck, it is commonly misdiagnosed as other thyroid neoplasms. Case Report. We report a case of cervical paraganglioma in a 55-year-old female. Patient was admitted to our clinic with goiter and neck pain. Thyroid ultrasonography revealed a 20 mm solitary, heterogeneous nodule located in the upper pole of left thyroid lobe. Fine needle aspiration cytology was nondiagnostic. She underwent left lobectomy and histopathology showed paraganglioma. Discussion. Cervical paragangliomas should be considered in the differential diagnosis of thyroid nodules.

Christ siemens touraine syndrome: A rare case report

Directory of Open Access Journals (Sweden)

N Retnakumari

2016-01-01

Full Text Available Christ-Siemens-Touraine (CST is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.

Tuberculous osteomyelitis affecting periodontium: A rare case report

Directory of Open Access Journals (Sweden)

Gaurav Bakutra

2015-01-01

Full Text Available Tuberculous lesions affecting periodontium are rare and seen as secondary infections localized to the soft tissues. With the advent of effective drug therapy, tuberculous lesions of the oral cavity have become rare. Involvement of the periodontium has seldomly been reported in the recent literature. We report a case of tuberculous osteomyelitis of mandible affecting periodontium leading to gingival recession and bone exposure in the mandibular premolar region in a 42-year-old female patient. The diagnosis was based on patient's medical and dental history, bacterial culture, clinical and radiographic examination, blood investigation, immunologic tests, histopathologic examination of the tissue specimen. Patient was already taking antitubercular chemotherapy prescribed by physician. Sequestrectomy and decortications were carried out to remove the affected bone. Healing was uneventful and there was no recurrence after 1½ year of follow-up. Antitubercular chemotherapy along with sequestrectomy and decortication are the treatment of choice for tuberculous osteomyelitic lesions affecting periodontium.

Christ Siemens Touraine syndrome: A rare case report.

Science.gov (United States)

Retnakumari, N; Varghese, Manuja; Kannan, V P

2016-01-01

Christ-Siemens-Touraine (CST) is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.

Bullosis Diabeticorum: Rare Presentation in a Common Disease

Directory of Open Access Journals (Sweden)

Vineet Gupta

2014-01-01

Full Text Available A 27-year-old African American male presented with a sudden onset of blisters. He had a past medical history of uncontrolled diabetes mellitus type I, diabetic vasculopathy, and neuropathy. The physical examination revealed nonerythematous skin denudations on both elbows and lateral aspect of arm bilaterally. Investigations which included skin biopsies confirmed the diagnosis of bullosis diabeticorum. The bullae were treated with hydrotherapy and healed with no complications in 4 weeks. We present this case to illustrate the rare occurrence of diabetic bulla in a diabetic patient especially with poor glycemic control. The case is also a reminder of the importance of diabetes screening in nondiabetic patients who are diagnosed with diabetic bulla.

Sampling rare fluctuations of discrete-time Markov chains

Science.gov (United States)

Whitelam, Stephen

2018-03-01

We describe a simple method that can be used to sample the rare fluctuations of discrete-time Markov chains. We focus on the case of Markov chains with well-defined steady-state measures, and derive expressions for the large-deviation rate functions (and upper bounds on such functions) for dynamical quantities extensive in the length of the Markov chain. We illustrate the method using a series of simple examples, and use it to study the fluctuations of a lattice-based model of active matter that can undergo motility-induced phase separation.

Intraoral hydatid cyst: A rare case report

Directory of Open Access Journals (Sweden)

Ravi Kiran Alaparthi

2015-01-01

Full Text Available "Hydatid" is a Greek word which means "a drop of water." Hydatid cysts occur in hydatid disease or echinococcosis, which is one of the most geographically prevalent zoonosis. This zoonotic infection in humans is mainly caused by infection by the larval stage of the dog tapeworm Echinococcus granulosus. This tapeworm infestation is widespread in sheep, goats, cattle, and dogs. This chronic disease is present worldwide among herding populations who live in close proximity to dogs and herd animals. It is a serious and potentially fatal condition and symptoms may occur a long time after the early infection. The most frequently affected organs are liver, lungs, followed by bones and brain, and extremely unusual occurrence has been found in the oral cavity, which was noticed in the present case. So hereby, we reveal a very rare case of intraoral hydatid cyst in a 20-year-old female patient.

Zosteriform Lichen Planus: case report of a rare variant of Lichen Planus

OpenAIRE

Kanthilatha Pai; Sathish Pai

2013-01-01

Since its original description by Devergie in 1854, several cases of linear lichen planus have been described in the literature, but there have been notably few cases of the more rare zosteriform lichen planus. Zosteriform lichen planus needs to be differentiated from linear lichen planus and other linear dermatoses. We present a case of Zosteriform Lichen planus for its rarity and briefly review literature.

Cerebral vasculitis and lateral rectus palsy - two rare central nervous system complications of dengue fever: two case reports and review of the literature.

Science.gov (United States)

Herath, H M M; Hewavithana, J S; De Silva, C M; Kularathna, O A R; Weerasinghe, N P

2018-04-19

Dengue fever is a common mosquito-borne viral illness with a clinical spectrum ranging from a simple febrile illness to potentially life-threatening complications such as dengue hemorrhagic fever and dengue shock syndrome. Dengue infection can affect many organs, including the central nervous system. The neurological manifestations reported in dengue infections are meningitis, encephalitis, stroke, acute disseminated encephalomyelitis, and Guillain-Barré syndrome. We report the cases of two interesting patients with confirmed dengue infection who presented with complications of possible central nervous system vasculitis and cranial nerve palsy. The first patient was a 53-year-old previously healthy Singhalese woman who developed acute-onset slurring of speech and ataxia with altered sensorium 1 day after recovery from a critical period of dengue hemorrhagic fever. Subsequent investigations revealed evidence of encephalopathy with brainstem ischemic infarctions. Her clinical picture was compatible with central nervous system vasculitis. She was treated successfully with intravenous steroids and had a full functional recovery. The second patient was a middle-aged Singhalese woman who had otherwise uncomplicated dengue infection. She developed binocular diplopia on day 4 of fever. An ocular examination revealed a convergent squint in the left eye with lateral rectus palsy but no other neurological manifestation. Central nervous system vasculitis due to dengue infection is a very rare phenomenon, and to the best of our knowledge, only one case of central nervous system vasculitis has been reported to date, in a patient of pediatric age. Cranial nerve palsy related to dengue infection is also rare, and only a few cases of isolated abducens nerve palsy have been reported to date. The two cases described in this report illustrate the rare but important central nervous system manifestations of dengue fever and support the fact that the central nervous system is one of the

A Rare Case of Metastatic Choriocarcinoma of Lung Origin

Directory of Open Access Journals (Sweden)

Parth Rali

2017-01-01

Full Text Available Choriocarcinoma is part of the spectrum of gestational trophoblastic disease that occurs in women of reproductive age. Although the most common metastatic site of choriocarcinoma is the lung, primary pulmonary choriocarcinoma is rare. To diagnose primary pulmonary choriocarcinoma, the patient should have no previous gynecologic malignancy, have elevated human chorionic gonadotropin, and have pathological confirmation of the disease excluding gonadal primary site of the tumor. Due to the paucity of data, there are no guidelines for treatment. Prognosis of this malignancy is extremely poor. We report a rare case of metastatic primary lung choriocarcinoma in a 69-year-old postmenopausal woman who was treated with combination of surgery, chemotherapy, and radiation. The patient had a good outcome and is doing well after 1-year follow-up.

Sirenomelia: a rare case of foetal congenital anomaly.

Science.gov (United States)

Dharmraj, Meena; Gaur, Sumitra

2012-10-01

Sirenomelia, alternatively known as 'mermaid syndrome' is a very rare congenital deformity in which the legs are fused together, giving them the appearance of the tail of a mermaid'. Other birth defects are always associated with sirenomelia, most commonly abnormalities of the kidneys, large intestines, and genitalia. The present case is a one of sirenomelia associated with an absent right kidney, mild left hydronephrosis, single umbilical artery, and severe oligohydramnios. We discuss the findings, relative to the present literature and related etiopathogenesis.

Isolated pulmonary candidiasis in a patient with diabetes mellitus: A rare case report.

Science.gov (United States)

Hakamifard, Atousa; Khorvash, Farzin; Raisi, Arash

2016-01-01

Fungal infections are as a cause of morbidity and mortality in immunocompromise patients. Because the respiratory tract is colonized with Candida, the presence of this agent in respiratory specimens makes the diagnosis of Candida pneumonia problematic. Candida pneumonia is a rare infection, and the majority of cases are secondary to hematogenous dissemination. Furthermore isolated Candida pneumonia originating from endotracheal inoculation is an extremely rare entity. We describe a case of isolated pulmonary candidiasis in the form multiple nodular lesions in a patient with long-term history of diabetes mellitus without evidence of fungemia or systemic involvement who responded to antifungal therapy.

Rare Infratentorial and Supratentorial Localization of Juvenile Angiofibroma: A Case Report.

Science.gov (United States)

Pašalić, Ivan; Trninić, Ines; Nemir, Jakob; Jednačak, Hrvoje; Žarković, Kamelija; Mrak, Goran

2016-01-01

Angiofibromas are rare tumors of the head and neck that mostly occur in the sphenopalatine region. We present a case of angiofibroma in a young male patient with an unusual and extremely rare localization, which to our knowledge has not been described before. It was situated in the tentorium and spread to the supratentorial and infratentorial regions. The patient initially presented with symptoms of increased intracranial pressure. After a diagnostic evaluation was done, the whole tumor was successfully removed using the supratentorial and infratentorial approach and the microsurgical technique. © 2016 S. Karger AG, Basel.

A rare case of recurrent pyogenic liver abscess since childhood: A case of Papillon-Lefèvre syndrome

Directory of Open Access Journals (Sweden)

Somak K Das

2013-01-01

Full Text Available Recurrent pyogenic liver abscess since childhood is an uncommon finding in clinical medicine. Papillon-Lefèvre syndrome (PLS is a rare disease characterized by skin lesions caused by palmar-plantar hyperkeratosis, and severe periodontal destruction involving both the primary and permanent dentitions. Till date, more than 200 cases have been reported worldwide. In addition to the skin and oral findings, patients may have immune suppression and an increased susceptibility to bacteria, associated with recurrent pyogenic infections of the skin. Pyogenic liver abscess is an uncommon presentation of this rare syndrome. We present a case of PLS presenting as recurrent pyogenic liver abscess since childhood.

A rare mitochondrial disorder: Leigh sydrome - a case report

Directory of Open Access Journals (Sweden)

Shrikhande Dhananjay Y

2010-09-01

Full Text Available Abstract Leigh syndrome is a rare progressive neurodegenerative, mitochondrial disorder of childhood with only a few cases documented from India. The clinical presentation of Leigh syndrome is highly variable. However, in most cases it presents as a progressive neurological disease with motor and intellectual developmental delay and signs and symptoms of brain stem and/or basal ganglia involvement. Raised lactate levels in blood and/or cerebrospinal fluid is noted. It is the neuroimaging, mainly the Magnetic Resonance Imaging showing characteristic symmetrical necrotic lesions in the basal ganglia and/or brain stem that leads to the diagnosis. Here, we report a case of 7 months old female child presenting to us with status epilepticus, delayed developmental milestones and regression of the achieved milestones suspected to be a case of neurodegenerative disorder, which on MRI was diagnosed as Leigh syndrome.

[Nephroblastoma and xeroderma pigmentosum: A rare association].

Science.gov (United States)

Lahlimi, F; Harif, M; Elhoudzi, J

2016-01-01

Xeroderma pigmentosum (XP) is a rare, genetically heterogeneous, autosomal recessive disorder, more common in cases of consanguinity. The basic defect underlying the clinical manifestations is a nucleotide excision repair defect leading to the defective repair of DNA damaged by ultraviolet (UV) radiation. XP is characterized by a high incidence of skin cancer on exposed regions. We report the case of a 5-year-old boy, followed for xeroderma pigmentosum since the age of 4 years. His sister also has the same anomaly. He presented an abdominal mass revealed by abdominal pain and vomiting. Radiological examinations revealed a nephroblastoma with lung metastases. He received primary chemotherapy for six cycles (vincristine, and actinomycin-d adriamycin), then surgery with ureteronephrectomy. Pathological examination of the nephrectomy specimen confirmed the diagnosis of Wilms tumor with a diffuse anaplastic component reaching 50%. The patient was treated according to the GFAOP stage III protocol, with high histological risk. The outcome was favorable but complicated by renal failure due to the toxicity of the treatment. He is currently in complete remission at 1 year from the end of treatment. The association of xeroderma pigmentosum and nephroblastoma is a rare combination. This case illustrates the problem of management of both severe and difficult conditions. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

PRIMARY MULTILOCULAR HYDATID CYST OF NECK : A RARE CASE REPORT

Directory of Open Access Journals (Sweden)

Deepak Ramraj

2015-02-01

Full Text Available Hydatid disease, also known as echinococcosis or hydatidosis , is an infectious disease caused by Echinococcus. Echinococcus granulosus is the most common Echinococcus species affecting human beings. It may affect any organ and tissue in the body, in particular the liver and lung. Musculoskeletal or soft tissue hyda tidosis accounts for about 0.5% 5% of all echinococcal infections in endemic areas, and is almost always secondary to the hepatic or pulmonary disease. Even in regions where echinococcosis is endemic, hydatidosis of cervicofacial region is extremely rare. Herein, we present exceptionally rare case in a 55 year old female with an unusual localization of primary multilocular hydatid cyst in the right supraclavicular region of the neck. A high index of suspicion is required to diagnose hydatid cyst in rare loc ations like this. Hydatid cyst should be considered in differential diagnosis of benign swellings of head and neck region, so that it can be managed during surgery to prevent acute anaphylaxis

Metastasis of Lung Adenocarcinoma to the Gingiva: A Rare Case Report

Directory of Open Access Journals (Sweden)

M. Rajini Kanth

2015-05-01

Full Text Available Metastatic tumors account for 1% of all oral malignancies. Metastasis to jaw bones is common, particularly in the mandible, rare in the oral soft tissues, and account for only 0.1% of oral malignancies. The majority of metastatic cases (70% reported in the literature have primary tumors located in the lung, breast, kidney, and colon. Metastasis is a biological complex process that involves detachment from the surrounding cells, regulation of cell motility, invasion, survival, proliferation, and evasion of the immune system. Clinical presentation of metastatic tumors is variable, which may create diagnostic dilemma or may lead to erroneous diagnosis. Metastatic tumors clinically mimic as dental infections. Metastasis to the oral soft tissue from lung cancer, especially gingiva is a rare condition. Metastasis to the gingiva can affect the oral function, speech, and nutrition. Most of the cases in the literature reported that lesion presented in oral soft tissues before the diagnosis of primary tumors. Here we report a case of 62-year-old male patient with metastasis from lung to the gingiva, where the metastasis was detected before primary tumor.

In situ follicular neoplasia/lymphoma: Three illustrative cases exemplifying unique disease presentations

Directory of Open Access Journals (Sweden)

Uma Sakhadeo

2012-01-01

Full Text Available We report three elderly patients with follicular lymphoma in situ (FLIS each highlighting a unique pattern of disease presentation and progression. The first patient had incidentally detected FLIS with peripheral blood spill and yet had an 11-year uneventful follow up. The second patient with an overt follicular lymphoma (FL developed high-grade transformation in jejunum with FLIS extensively involving the Payers patches. The third patient had a FLIS but that qualified as higher grade and was treated in spite of lack of overt FL mainly because of higher grade and patient subsequently did develop overt FL. The first case of typical FLIS confirms that peripheral blood spill does not connote poor prognosis in FLIS, the second case illustrates that FLIS may colonize mucosa-associated lymphoid tissue as part of homing in process of a disseminated FL and the third case validates the aggressive nature of high-grade FLIS.

A rare case of extra-nasopharyngeal angiofibroma of the septum in a female child.

Science.gov (United States)

Singh, G B; Shukla, S; Kumari, P; Shukla, I

2018-02-01

Extra-nasopharyngeal angiofibroma is a rare but distinct clinical entity, different from juvenile angiofibroma. This clinical record elucidates the only case of extra-nasopharyngeal angiofibroma arising from the septum in a female child, who presented with epistaxis. The histopathological diagnosis was confirmed by immunohistochemistry, and the case was managed surgically with no recurrence. In a female paediatric patient presenting with epistaxis, extra-nasopharyngeal angiofibroma (of the inferior turbinate) is a rare albeit important differential diagnosis, as it challenges the hormonal theory of angiofibroma aetiopathogenesis.

A rare case of traumatic chylothorax after blunt thoracic trauma

Directory of Open Access Journals (Sweden)

Spasić Marko

2017-01-01

Full Text Available Introduction. Chylothorax is an accumulation of chyle in the pleural cavity due to a disruption of the thoracic duct. Traumatic chylothoraces are usually a result of a penetrating trauma and disruption of the thoracic duct, but blunt traumatic chylothorax is a rare condition. The aim of this paper is to present a rare case of traumatic chylothorax after blunt thoracic trauma. Case Outline. We present a case of traumatic chylothorax after blunt thoracic trauma in a patient injured in a motor vehicle accident. The patient had a right-sided fracture of rib XI, hydropneumothorax, lung contusion, and signs of pneumomediastinum. We performed thoracic drainage, but a few days later, according to the increase of amount of the fluid daily drained, and the confirmation of laboratory findings of the analyzed fluid, we made a diagnosis of chylothorax and the patient underwent a thoracotomy, where we sutured the thoracic duct. Conclusion. Chylothorax should be considered in patients after chest trauma if they develop a milky pleural effusion. Analysis of pleural fluid and level of triglycerides is important for the diagnosis and treatment of chylothorax. [Project of the Serbian Ministry of Education, Science and Technological Development, Grant no. III41007

A rare case of malignant triton tumor in the cerebellopontine angle.

Science.gov (United States)

Gong, Li; Liu, Xiao-Yan; Zhang, Wen-Dong; Han, Xiu-Juan; Yao, Li; Zhu, Shao-Jun; Lan, Miao; Li, Yan-Hong; Zhang, Wei

2012-04-19

Malignant triton tumor (MTT) is defined as malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. Intracranial MTT is extremely rare, and only four cases have been reported in the literature. Here, we report a case of MTT occurring in the cerebellopontine angle, and describe its histopathological characteristics, immunohistochemical features, and prognosis. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1336227313684480.

Waardenburg syndrome: A rare genetic disorder, a report of two cases.

Science.gov (United States)

Kumar, Sudesh; Rao, Kiran

2012-05-01

Waardenburg syndrome (WS) is a rare genetic disorder. Patients have heterochromia or eyes with iris of different color, increased inter-canthal distance, distopia canthorum, pigmentation anomalies, and varying degree of deafness. It usually follows autosomal dominant pattern. In this report, two cases have been discussed but no familial history of WS has been found. Counseling of the patient is necessary and cases of irreversible deafness have been treated.

A rare case of Moebius sequence

Directory of Open Access Journals (Sweden)

Abhishek Kulkarni

2012-01-01

Full Text Available We report a case of an 18-year-old male who presented with watering and inability to close the left eye completely since 6 months and inability to move both eyes outward and to close the mouth since childhood. Ocular, facial, and systemic examination revealed that the patient had bilateral complete lateral rectus and bilateral incomplete medial rectus palsy, left-sided facial nerve paralysis, thickening of lower lip and inability to close the mouth, along with other common musculoskeletal abnormalities. This is a typical presentation of Moebius syndrome which is a very rare congenital neurological disorder characterized by bilateral facial and abducens nerve paralysis. This patient had bilateral incomplete medial rectus palsy which is suggestive of the presence of horizontal gaze palsy or occulomotor nerve involvement as a component of Moebius sequence.

Congenital epulides: A rare case report

Directory of Open Access Journals (Sweden)

Saurabh Kumar

2014-01-01

Full Text Available Congenital epulis (CE or "Granular cell epulis" also previously termed as "Neumann′s tumor" is a benign growth arising from the mucosa of the gingiva, typically seen as a mass protruding from the infant′s mouth, often interfering with respiration and feeding. These tumors generally present as a single mass arising from the upper alveolus. We report a rare case of two congenital epulides arising from the maxillary and mandibular alveolus in a day old female infant, which was surgically excised, allowing for early initiation of breast feeding. The tumor cells stained negative for S100 protein hence differentiating from other granular cell tumors. The clinical presentation, differential diagnosis in regard to the various neonatal oral swellings and the management of CE has been discussed.

Acute amebic appendicitis: Report of a rare case

Directory of Open Access Journals (Sweden)

Singh Naorem

2010-10-01

Full Text Available Acute appendicitis of amebic origin is considered a rare cause of acute appendicitis. We report a case of amebic appendicitis presenting with fever, severe pain in the right lower quadrant of the abdomen and rebound tenderness. Lab investigations revealed neutrophilic leukocytosis. The patient underwent appendectomy. Histopathological examination revealed numerous Entameba histolytica trophozoites in the mucosa of the appendix. Acute appendicitis of amebic origin does not appear frequently. Appendicular amebiasis can give the clinical features of acute appendicitis and should be treated accordingly.

Lean practices for quality results: a case illustration.

Science.gov (United States)

Hwang, Pauline; Hwang, David; Hong, Paul

2014-01-01

Increasingly, healthcare providers are implementing lean practices to achieve quality results. Implementing lean healthcare practices is unique compared to manufacturing and other service industries. The purpose of this paper is to present a model that identifies and defines the lean implementation key success factors in healthcare organisations. The model is based on an extant literature review and a case illustration that explores actual lean implementation in a major USA hospital located in a Midwestern city (approximately 300,000 people). An exploratory/descriptive study using observation and follow-up interviews was conducted to identify lean practices in the hospital. Lean practice key drivers include growing elderly populations, rising medical expenses, decreasing insurance coverage and decreasing management support. Effectively implementing lean practices to increase bottom-line results and improve organisational integrity requires sharing goals and processes among healthcare managers and professionals. An illustration explains the model and the study provides a sound foundation for empirical work. Practical implications are included. Lean practices minimise waste and unnecessary hospital stays while simultaneously enhancing customer values and deploying resources in supply systems. Leadership requires clear project targets based on sound front-end planning because initial implementation steps involve uncertainty and ambiguity (i.e. fuzzy front-end planning). Since top management support is crucial for implementing lean practices successfully, a heavyweight manager, who communicates well both with top managers and project team members, is an important success factor when implementing lean practices. Increasingly, green orientation and sustainability initiatives are phrases that replaced lean practices. Effective results; e.g. waste reduction, employee satisfaction and customer values are applicable to bigger competitive challenges arising both in specific

[Partial facial duplication (a rare diprosopus): Case report and review of the literature].

Science.gov (United States)

Es-Seddiki, A; Rkain, M; Ayyad, A; Nkhili, H; Amrani, R; Benajiba, N

2015-12-01

Diprosopus, or partial facial duplication, is a very rare congenital abnormality. It is a rare form of conjoined twins. Partial facial duplication may be symmetric or not and may involve the nose, the maxilla, the mandible, the palate, the tongue and the mouth. A male newborn springing from inbred parents was admitted at his first day of life for facial deformity. He presented with hypertelorism, 2 eyes, a tendency to nose duplication (flatted large nose, 2 columellae, 2 lateral nostrils separated in the midline by a third deformed hole), two mouths and a duplicated maxilla. Laboratory tests were normal. The cranio-facial CT confirmed the maxillary duplication. This type of cranio-facial duplication is a rare entity with about 35 reported cases in the literature. Our patient was similar to a rare case of living diprosopus reported by Stiehm in 1972. Diprosopus is often associated with abnormalities of the gastrointestinal tract, the central nervous system, the cardiovascular and respiratory systems and with a high incidence of cleft lip and palate. Surgical treatment consists in the resection of the duplicated components. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

A giant occipital encephalocele with spontaneous hemorrhage into the sac: A rare case report

OpenAIRE

Nath, H. D.; Mahapatra, A. K.; Borkar, S. A.

2014-01-01

In giant encephalocele, head size is smaller than the encelphalocele. Occipital encephalocele is the commonest of all encephalocele. In our case, there was rare association with giant encephalocele with old hemorrhage in the sac. This was a unique presentation. In world literature, there was rare association with giant encephalocele with hemorrhage.

A rare case of acquired methemoglobinemia associated with alkaptonuria.

Science.gov (United States)

Isa, Yasuki; Nihei, Shun-ichi; Irifukuhama, Yuna; Ikeda, Tomoya; Matsumoto, Hiroyuki; Nagata, Keiji; Harayama, Nobuya; Aibara, Keiji; Kamochi, Masayuki

2014-01-01

We herein present a rare case of acquired methemoglobinemia associated with alkaptonuria. Alkaptonuria is a congenital error of metabolism caused by the deficiency of homogentisic acid oxidase, which subsequently results in the accumulation of homogentisic acid (HGA) in body tissues. As renal dysfunction progresses, the level of HGA excretion in the urine decreases and the blood concentration of HGA increases. HGA oxidizes oxyhemoglobin to methemoglobin, which can induce multiple organ failure accompanied by tissue hypoxia, intravascular hemolysis and metabolic acidosis. The mortality of this disease is high when alkaptonuria is associated with the presence of methemoglobinemia; therefore, treatment should be carefully planned in such cases.

A rare case of allergic bronchopulmonary mycosis caused by Alternaria alternata.

NARCIS (Netherlands)

Chowdhary, A.; Agarwal, K.; Randhawa, H.S.; Kathuria, S.; Gaur, S.N.; Najafzadeh, M.J.; Roy, P.; Arora, N.; Khanna, G.; Meis, J.F.G.M.

2012-01-01

A rare case of allergic bronchopulmonary mycosis (ABPM), caused by Alternaria alternata, is reported in an immunocompetent resident of Delhi. Her complaints included a generalized, urticarial skin rash and occasional pain in the right lower chest. Her differential count showed eosinophils, 22%;

A rare case of a retroperitoneal enterogenous cyst with in-situ adenocarcinoma

Directory of Open Access Journals (Sweden)

Woodland James G

2007-10-01

Full Text Available Abstract Background Retroperitoneal enterogenous cysts are uncommon and adenocarcinoma within such cysts is a rare complication. Case presentation We present the third described case of a retroperitoneal enterogenous cyst with adenocarcinomatous changes and only the second reported case whereby the cyst was not arising from any anatomical structure. Conclusion This case demonstrates the difficulties in making a diagnosis as well as the importance of a multi-disciplinary approach, and raises further questions regarding post-operative treatment with chemotherapy.

Isolated pulmonary candidiasis in a patient with diabetes mellitus: A rare case report

Directory of Open Access Journals (Sweden)

Atousa Hakamifard

2016-01-01

Full Text Available Fungal infections are as a cause of morbidity and mortality in immunocompromise patients. Because the respiratory tract is colonized with Candida, the presence of this agent in respiratory specimens makes the diagnosis of Candida pneumonia problematic. Candida pneumonia is a rare infection, and the majority of cases are secondary to hematogenous dissemination. Furthermore isolated Candida pneumonia originating from endotracheal inoculation is an extremely rare entity. We describe a case of isolated pulmonary candidiasis in the form multiple nodular lesions in a patient with long-term history of diabetes mellitus without evidence of fungemia or systemic involvement who responded to antifungal therapy.

Ingested razor blades within the appendix: A rare case report

Directory of Open Access Journals (Sweden)

Jason Cui

Full Text Available Introduction: Foreign body ingestion is a common clinical presentation with less than 1% of the cases requiring surgical intervention. In this report, we present a rare case of razor blades lodged in the appendix as a result of intentional ingestion. Presentation of case: A 25 year old male prisoner presented to our hospital with persistent right iliac fossa pain after razor blade ingestion. After 5 days of conservative management, there was no sign of transition on serial X-Rays. Laparoscopy with intraoperative image intensification confirmed the presence of the razor blades in the appendix and appendicectomy was subsequently performed without complications. Discussion: Most ingested objected with diameter less than 2.5 cm and length less than 6 cm can pass through the gastrointestinal tract spontaneously in less than one week. The entry of foreign objects into the appendix is thought to be due to relative low motility of the caecum, the dependent position of the appendix and the size of the appendiceal orifice. Radiographic localisation to the appendiceal lumen was complicated by metallic artefact, but was consistent with failure to transit. Appendicectomy was felt to be the safest mode of retrieval. Conclusion: Ingested foreign body lodged in the appendix is a rare event. Once the exact location is confirmed, a simple laparoscopic appendicectomy can be performed to facilitate the removal. Keywords: Appendicitis, Laparoscopy, Appendicectomy, Foreign body ingestion, Razor blades, Case report

Avoidant restrictive food intake disorder: an illustrative case example.

Science.gov (United States)

Bryant-Waugh, Rachel

2013-07-01

Avoidant/restrictive food intake disorder (ARFID) is a new diagnostic category in DSM-5. Although replacing Feeding Disorder of Infancy or Early Childhood, it is not restricted to childhood presentations. In keeping with the broader aim of revising and updating criteria and text to better reflect lifespan issues and clinical expression across the age range, ARFID is a diagnosis relevant to children, adolescents, and adults. This case example of a 13-year old boy with ARFID illustrates key issues in diagnosis and treatment planning. The issues discussed are not exhaustive, but serve as a guide for central diagnostic and treatment issues to be considered by the clinician. It is anticipated that the inclusion of specific criteria for ARFID as a category within Feeding and Eating Disorders in DSM-5 will stimulate research into its typology, prevalence, and incidence in different populations and facilitate the development of effective, evidence-based interventions for this patient group. Copyright © 2013 Wiley Periodicals, Inc.

Enterovesical fistula, a rare complication of Meckel’s diverticulum: A case report

Directory of Open Access Journals (Sweden)

Bourguiba M.A.

2017-01-01

Conclusion: Vesico-diverticular fistula resulting from a perforated Meckel's diverticulum is a rare complication. To our knowledge, this is only the fourth reported case which is not associated to inflammatory bowel disease.

A rare case of de novo gigantic ovarian abscess within an endometrioma.

Science.gov (United States)

Hameed, Aisha; Mehta, Vaishali; Sinha, Prabha

2010-06-01

We are reporting a rare case of de novo ovarian abscess in an endometrioma. Ovarian abscess within an endometrioma is a rare gynecological problem, but de novo abscess in the endometrioma is even rarer. Most of the ovarian abscesses develop in the endometriomas following interventions, e.g., aspiration, pelvic surgery, and oocyte retrieval. We are presenting a case of a spontaneous giant abscess in a large ovarian cyst in a nulliparous woman who presented with acute abdomen. Patient was treated in a district general hospital with multidisciplinary approach. Thirteen liters of the pus were drained. She has had a sub total (supra cervical) hysterectomy and bilateral salpingo-oophorectomy (BSO) performed. Histology of the abscess wall confirmed endometriotic nature of the cyst. Patient made an uneventful recovery and was discharged home on the 14th postoperative day. This case highlights that endometrioma and its complication can present as a surgical emergency and should be dealt as one.

Processes for the production of rare earths from monazite (Paper No. 36)

International Nuclear Information System (INIS)

Murthy, T.K.S.

1979-01-01

A few typical cases are briefly described to illustrate different methods available for rare earth concentration and separation from mixed rare earths chloride obtained for monazite. In the case of cerium, rare earths chloride mixture is treated with sodium sulphate to precipitate rare earths as double sulphates from which hydroxide cake is prepared. The cake is dried to oxidise cerium. Trivalent rare earths are selectively leached and the product is treated with HNO 3 . The resulting ceric nitrate solution is purified by liquid-liquid extraction using TBP. The scrubbed extract is reduced with H 2 O 2 and cerous nitrate is recovered by evaporation. Lanthanum is first concentrated by selective precipitation of hydroxides from rare earths chloride using air-ammonia mixture. The hydroxyde cake is dissolved in HNO 3 and NH 4 NO 3 to get the double nitrate which is subjected to counter current crystallisation for purification. Europium is present at a concentration of 0.01% in the rare earths chloride. It is concentrated in several steps by liquid-liquid extraction using di-2-ethyl hexyl phosphoric acid. The product is purified by selective reduction and europium is obtained as europium sulphate. In the same solvent extraction process samarium and gadolinium are also concentrated to about 25%. They are further upgraded to above 90% purity by repetion of liquid-liquid extraction technique. Cerium, lanthanum and europium obtained by the above processes analyse > 99% as oxides. (M.G.B.)

A giant occipital encephalocele with spontaneous hemorrhage into the sac: A rare case report

Science.gov (United States)

Nath, H. D.; Mahapatra, A. K.; Borkar, S. A.

2014-01-01

In giant encephalocele, head size is smaller than the encelphalocele. Occipital encephalocele is the commonest of all encephalocele. In our case, there was rare association with giant encephalocele with old hemorrhage in the sac. This was a unique presentation. In world literature, there was rare association with giant encephalocele with hemorrhage. PMID:25685207

Digital test assembly of truck parts with the IMMA-tool--an illustrative case.

Science.gov (United States)

Hanson, L; Högberg, D; Söderholm, M

2012-01-01

Several digital human modelling (DHM) tools have been developed for simulation and visualisation of human postures and motions. In 2010 the DHM tool IMMA (Intelligently Moving Manikins) was introduced as a DHM tool that uses advanced path planning techniques to generate collision free and biomechanically acceptable motions for digital human models (as well as parts) in complex assembly situations. The aim of the paper is to illustrate how the IPS/IMMA tool is used at Scania CV AB in a digital test assembly process, and to compare the tool with other DHM tools on the market. The illustrated case of using the IMMA tool, here combined with the path planner tool IPS, indicates that the tool is promising. The major strengths of the tool are its user friendly interface, the motion generation algorithms, the batch simulation of manikins and the ergonomics assessment methods that consider time.

Synovial sarcoma of primary bone origin: a rare case in a rare site with atypical features

International Nuclear Information System (INIS)

Jung, Seung Chai; Choi, Jung-Ah; Lee, Joon Woo; Kang, Heung Sik; Chung, Jin-Haeng; Oh, Joo Han

2007-01-01

Synovial sarcoma of bone origin is extremely rare and difficult to diagnose. We present a case in which the lesion arose in the cortex of the distal tibia. It showed heterogeneous intermediate signal intensity on T1-weighted images and heterogeneous intermediate to low signal intensity on T2-weighted images with heterogeneous contrast enhancement on MRI. The lesion was confirmed as synovial sarcoma using a combination of histological and molecular genetic studies. (orig.)

Aneurysmal bone cyst of maxillary alveolus: A rare case report

Directory of Open Access Journals (Sweden)

Subhas Chandra Debnath

2016-01-01

Full Text Available Aneurysmal bone cyst (ABC is a nonneoplastic rare pathologic entity of the jaws. Its locally aggressive nature and high recurrence rate after curettage make surgical resection a better treatment option. Here, we present a case of ABC of maxillary alveolus and its management by alveolectomy followed by white head varnish pack application in the surgical defect.

Aneurysmal bone cyst of maxillary alveolus: A rare case report

Science.gov (United States)

Debnath, Subhas Chandra; Adhyapok, Apurba Kumar; Hazarika, Kriti; Malik, Kapil; Vatsyayan, Ashutosh

2016-01-01

Aneurysmal bone cyst (ABC) is a nonneoplastic rare pathologic entity of the jaws. Its locally aggressive nature and high recurrence rate after curettage make surgical resection a better treatment option. Here, we present a case of ABC of maxillary alveolus and its management by alveolectomy followed by white head varnish pack application in the surgical defect. PMID:27041915

Neurological manifestations in speech after snake bite: A rare case ...

African Journals Online (AJOL)

Neurological manifestations in speech after snake bite: A rare case. D Vir, D Gupta, M Modi, N Panda. Abstract. No Abstract. Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT · http://dx.doi.org/10.4314/pamj.v4i1.53597 · AJOL African Journals Online. HOW TO ...

Chondroblastoma of the thoracic spine: a rare location. Case report with radiologic-pathologic correlation

Energy Technology Data Exchange (ETDEWEB)

Venkatasamy, A. [University Hospital of Strasbourg, Department of Radiology, Strasbourg (France); Chenard, M.P. [University Hospital of Strasbourg, Department of Pathology, Strasbourg (France); Massard, G. [University Hospital of Strasbourg, Department of Thoracic Surgery, Strasbourg (France); Steib, J.P. [University Hospital of Strasbourg, Department of Spine Surgery, Strasbourg (France); Bierry, G. [University Hospital of Strasbourg, Department of Radiology, Strasbourg (France); University Hospital of Strasbourg, Department of Pathology, Strasbourg (France); University Hospital of Strasbourg, Department of Thoracic Surgery, Strasbourg (France); University Hospital of Strasbourg, Department of Spine Surgery, Strasbourg (France)

2017-03-15

Chondroblastoma is a rare benign cartilage neoplasm that arises from the appendicular skeleton in the vast majority of the cases (80%). Chondroblastoma of the spine is an even more rare condition (30 cases reported), and vertebral chondroblastomas, unlike chondroblastomas of the extremities, present with the appearance of an aggressive tumor on CT and MR imaging and occur at least a decade later. Even though vertebral chondroblastomas are very uncommon tumors, they should nonetheless be included in the differential diagnosis when encountered with an aggressive vertebral mass, and a histological confirmation should be performed. We present a case of chondroblastoma of the thoracic spine of a 27-year-old female for which detailed radiologic-pathologic correlation was obtained. (orig.)

Bilateral sertoli-leydig cell tumor of the ovary: A rare case report

OpenAIRE

Alam Kiran; Maheshwari Veena; Rashid Seema; Bhargava Shruti

2009-01-01

Sertoli leydig cell tumors also known as arrhenoblastoma, are a rare member of the sex cord-stromal tumor group of ovarian and testicular cancers, comprising less than 1% of all ovarian tumors, which occur in young adults and are almost always unilateral. We hereby report a case of a 17-year-old female presenting with a short history of irregular menses and an abdominal lump, which was histologically proven to be a bilateral sertoli leydig cell tumor of the ovary, an exceptionally rare...

Case report of a rare dermatosis in pregnancy: impetigo herpetiformis.

Science.gov (United States)

Ulubay, Mustafa; Keskin, Uğur; Fidan, Ulas; Çiçek, Ali Fuat; Çalışkan, Ercan; Karaca, Rıza Efendi; Fıratlıgil, Fahri Burçin; Ergün, Ali

2015-02-01

Impetigo herpetiformis (IH) is a very rare type of dermatosis seen in pregnancy. According to the published work, IH during pregnancy is associated with the risk of stillbirth, and obstetric management in such cases is very important. Early recognition is important to reduce both maternal and fetal morbidity. We present a case of IH resistant to corticosteroid therapy in a 27-year-old pregnant woman where the pregnancy was terminated by the induction of labor. © 2014 The Authors. Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology.

Gastric Volvulus: A Rare Entity Case Report and Literature Review

Science.gov (United States)

Akhtar, Aisha; Sheikh, Abdul Ahad E; Sheikh, Abu Baker; Perisetti, Abhilash

2018-01-01

Gastric volvulus is a rare entity defined as an abnormal rotation of the stomach around itself. It is a diagnosis of exclusion; the clinical index of suspicion is always low and is mostly diagnosed on imaging or on the surgery table. When it occurs, it is an emergency due to the risk of strangulation and consequent gangrene of the stomach. Mesentero-axial (MA) gastric volvuli constitute one-third of all cases. Here, we are present an interesting case of acute MA gastric volvulus diagnosed with imaging and treated subsequently. PMID:29755908

A rare case of malignant triton tumor in the cerebellopontine angle

Directory of Open Access Journals (Sweden)

Gong Li

2012-04-01

Full Text Available Abstract Malignant triton tumor (MTT is defined as malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. Intracranial MTT is extremely rare, and only four cases have been reported in the literature. Here, we report a case of MTT occurring in the cerebellopontine angle, and describe its histopathological characteristics, immunohistochemical features, and prognosis. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1336227313684480

Horizontal traumatic laceration of the pancreas head: A rare case report

OpenAIRE

Nanashima, Atsushi; Imamura, Naoya; Tsuchimochi, Yuki; Hamada, Takeomi; Yano, Kouichi; Hiyoshi, Masahide; Fujii, Yoshiro; Kawano, Fumiaki; MitsuruTamura,

2017-01-01

Introduction: This case report is intended to inform acute care surgeons about treating rare horizontal laceration of the pancreas head caused by blunt trauma. Case presentation: A 57-year-old woman who sustained blunt abdominal trauma during a car crash was transported to the emergency center of our hospital with unstable vital signs due to hemorrhagic shock. Computed tomography showed transection of the pancreas head and massive intra-abdominal hemorrhage. She was referred for emergency ...

Family Therapy Perspectives on Anxiety Manifestation in Career Counseling: A Case Illustration with an Adolescent

Science.gov (United States)

Rochat, Shékina

2018-01-01

Little is known about the origins of anxiety manifested in the career counseling process. Through a case illustration, this article highlights the appropriateness of using functional family therapy (FFT) principles in career counseling sessions to assess the family dynamics involved in this issue. The discussion emphasizes seven suggestions: (1)…

Rare case of Primary Pulmonary Extranodal Non-Hodgkin’s Lymphoma in a Patient with Sjogrens Syndrome: Role of FDG-PET/CT in the Initial Staging and Evaluating Response to Treatment

Directory of Open Access Journals (Sweden)

Gonca G. Bural

2012-12-01

Full Text Available A 64-year old woman with a long standing Sjogren’s syndrome was undergoing evaluation for renal transplant surgery when two pulmonary opacities were detected on chest CT. Subsequent biopsy revealed extranodal marginal B-cell non-Hodgkin’s lymphoma (NHL. An FDG-PET/CT scan was then performed which demonstrated isolated FDG avid pulmonary involvement. After therapy, FDG-PET/CT scans showed good response to treatment with near complete resolution of FDG avidity. This rare case illustrates the rare pulmonary manifestation of extranodal lymphoma in a patient with Sjogren’s syndrome and emphasizes the value of FDG PET/CT in the initial staging and evaluation of response to treatment, which has not previously been published. (MIRT 2012;21:117-120

Rare earth metals for automotive exhaust catalysts

International Nuclear Information System (INIS)

Shinjoh, Hirohumi

2006-01-01

The usage of rare earth metals for automotive exhaust catalysts is demonstrated in this paper. Rare earth metals have been widely used in automotive catalysts. In particular, three-way catalysts require the use of ceria compounds as oxygen storage materials, and lanthana as both a stabilizer of alumina and a promoter. The application for diesel catalysts is also illustrated. Effects of inclusion of rare earth metals in automotive catalysts are discussed

Elephantiasis nostras verrucosa: a rare complication of lower limb lymphoedema.

Science.gov (United States)

Kar Keong, Neoh; Siing Ngi, Angelina Tang; Muniandy, Pubalan; Voon Fei, Wong

2017-08-28

We reported a case of a 49-year-old man who had chronic lymphoedema of bilateral lower limbs for 30 years, but he did not seek treatment. His disease was complicated with irreversible changes of elephantiasis nostras verrucosa and had recurrent admissions due to infection. He was not keen for surgical intervention. This case report illustrated a rare and non-reversible complication of a common skin disease, lymphoedema and also the importance of identifying and treating it early. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Hereditary hypohidrotic ectodermal dysplasia: report of a rare case.

Science.gov (United States)

Paramkusam, Geetha; Meduri, Venkateswarlu; Nadendla, Lakshmi Kavitha; Shetty, Namratha

2013-09-01

Hereditary Hypohidrotic Ectodermal Dysplasia (HHED), an X-linked, recessive, Mendelian character, is seen usually in males and it is inherited through female carriers. It is characterised by congenital dysplasia of one or more ectodermal structures and it is manifested by hypohidrosis, hypotrichosis and hypodontia. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Here, we are presenting a rare case of HHED in a 19 year female with classic features of this condition.

Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: Two Unusual Presentations of a Rare Tumor

Directory of Open Access Journals (Sweden)

E. C. Castro

2012-01-01

Full Text Available Only few cases of primary renal Ewing's sarcoma have been reported in the literature to date. We present here two cases of renal ES/PNET with an uncanny presentation. The first case was discovered after the patient presented clinically with irradiating flank pain, mimicking the pain related with kidney stones. The second case had clinical presentation of pulmonary thromboembolism after the patient was involved in an automobilist accident. The tumors were mainly composed of small blue cells which by immunohistochemical were positive for neural markers, and FISH revealed the translocation 22q12 for the EWSR1 gene. The diagnosis of renal primitive neuroectodermal tumor/EWING tumor is very rare and usually involves several different diagnostic techniques. The differential diagnosis is usually broad with frequent overlapping features between the entities. The cases presented in this paper illustrated the difficulties with which routine anatomical pathologist is faced when dealing with rare renal poorly differentiated neoplasm in adults.

Non-syndromic multiple keratocyst odontogenic tumor: A rare case report

Directory of Open Access Journals (Sweden)

Abhijeet Alok

2015-01-01

Full Text Available Keratocystic odontogenic tumors (KCOTs are one of the most frequent features of nevoid basal cell carcinoma syndrome (NBCCS. The condition is linked with mutation in the PTCH gene. Partial expression of the gene may result in occurrence of multiple recurring odontogenic keratocysts (OKCs. Although KCOTs are common in clinical practice, simultaneous occurrence of multiple cysts in both the maxilla and mandible of a patient is rare. These patients have early propensity to develop multiple neoplasms like basal cell carcinoma and medulloblastoma. Hence, early diagnosis and treatment is of utmost importance in reducing the severity of the long-term sequelae of NBCCS. We report a rare case of multiple KCOTs in a non-syndromic male patient, with emphasis on its diagnosis, radiographic features, and treatment.

Facial Localization of Malignant Chondroid Syringoma: A Rare Case Report

Directory of Open Access Journals (Sweden)

Deniz Tural

2013-01-01

Full Text Available First described by Hirsch and Helwig in 1961, chondroid syringomas (CSs are rare, benign tumors of the skin arising from the eccrine sweat glands with tumor differentiation in the epithelial and mesenchymal tissues. They most commonly occur in the head and neck, although they may be also found in the axilla, trunk, limbs, and genitalia. The incidence of CS is <0.01% of all primary skin tumors. Malingnant chondroid syringomas (MCS, which are also called malignant mixed tumors of the skin, are extremely uncommon. MCSs commonly involve the limbs and rarely head and neck. In this article, we present a case of malignant chondroid syringoma localized in the face at the left nasolabial region in the light of literature review.

Co-occurrence of intraoral hemangioma and port wine stain: A rare case

Directory of Open Access Journals (Sweden)

Prasanna Kumar Rao

2012-01-01

Full Text Available Hemangiomas are neoplastic proliferations of endothelial cells, characterized by a period of growth after birth, and eventual spontaneous involution. The course can be uneventful with spontaneous resolution; or it may be marked by complications such as infection, bleeding, ulceration, visual defects and feeding difficulties. Apart from these, rare life-threatening complications such as congestive heart failure and consumption coagulopathy may also be seen. Although hemangiomas commonly occur in the head and neck region, intraoral occurrence is relatively rare. A port wine stain is defined as a macular telangiectatic patch which is present at birth and remains throughout life. They may be localized or extensive, affecting a whole limb. This article reports a rare case of co-occurrence of port wine stain with intraoral hemangioma.

Leiomyoma originating from axilla: A rare case report and differential diagnosis.

Science.gov (United States)

Kim, Ho Jun; Baek, Sang Oon; Rha, Eun Young; Lee, Jun Yong; Han, Hyun Ho

2016-07-01

Leiomyoma is a form of benign tumor originated in hypertrophy of the smooth muscles, which is most prevalent in the uterus and gastrointestinal tract. However, Leiomyoma originating from smooth muscle at the vessels lying on deep soft tissue is very rare. Our case was a rare case of leiomyoma originating from the axillary region, which was initially diagnosed as a fibroadenoma on radiological examination. The mass was separated from surrounding tissues and totally resected. Pathologically, hematoxylin-eosin-stained biopsy tissue showed the typical findings of leiomyoma. Postoperative follow-up observation was done for 1 year, without any complications or recurrence. Notably, a leiomyoma in the axillary region is difficult to differentiate from other benign or malignant tumors on preoperative radiological examinations such as ultrasonography or computed tomgraphy. Therefore, when an indefinite asymptomatic mass that is not lymphadenopathy or common benign tumor is identified in the axillary region, leiomyoma can be considered as one of the differential diagnoses.

Isolated peritoneal hydatidosis clinically mimicking ovarian tumor: A rare case report

Directory of Open Access Journals (Sweden)

Pradhan M Pagaro

2014-01-01

Full Text Available Hydatid cyst disease is rare and it is a parasitic infection where humans accidentally get infected by ingesting larval forms of parasite whereas, the definitive hosts are dog. The common sites of hydatid cyst are liver, lungs, spleen. Unusual sites of the hydatid cyst is reported in subcutaneous tissue of anterior abdominal wall, peritoneum. We report an unusual form of the primary hydatid cyst disease involving peritoneum in a 65-year-old female, presenting as swelling in the abdomen since 3 months. Sonography revealed a cystic mass and diagnosis of ovarian tumor was considered. The Cancer Antigen 125 (CA--125, an ovarian malignant marker was normal. Exploratory laprotomy was carried out. Cytological examination, gross, and the histopathological findings suggested the diagnosis of hydatid cyst disease involving only peritoneum. Primary isolated hydatidosis involving peritoneum is very rare and only few cases have been reported. Moreover, it mimics other tumors of the abdomen like in our case we considered it as an ovarian tumor.

Intestinal Duplication Cyst presenting as Volvulus: A rare case report with Review of Literatures

Directory of Open Access Journals (Sweden)

Prasan Kumar Hota

2015-03-01

Full Text Available Intestinal duplication cyst is a rare congenital anomaly occurring any where along the alimentary tract, from mouth to anus. They can present with numerous complications like perforation, bleeding or intestinal obstruction. We report a rare case  of intestinal duplication cyst of ileum with acute intestinal obstruction due to volvulus with review of literature.

Cutaneous collagenous vasculopathy: A rare case report

Directory of Open Access Journals (Sweden)

Kinjal Deepak Rambhia

2016-01-01

Full Text Available Cutaneous collagenous vasculopathy (CCV is a distinct, rare, and underdiagnosed condition. We report a case of CCV in a 50-year-old woman presenting as asymptomatic, erythematous to hyperpigmented nonblanchable macules over both the lower extremities. The clinical differential diagnosis of the lesions was pigmented purpuric dermatoses (Schamberg's purpura and cutaneous small vessel vasculitis. Histology of the lesions revealed dilated superficial dermal vessels with abundant pink hyaline material in the vessel wall, which stained with periodic acid Schiff stain. The patient was diagnosed as CCV. This condition remains largely underdiagnosed and is commonly mistaken for pigmented purpuric dermatosis or generalized essential telangiectasia. Emphasis on the differentiation of CCV from its clinical and histological mimicks is made.

A Rare Case of Epiphyseal Chondromyxoid Fibroma of the Proximal Tibia

International Nuclear Information System (INIS)

Choi, Yun Sun; Kim, Byoung Suck; Lee, Seok Hoon; Song, Baek Yong; Park, Yong Koo

2011-01-01

Chondromyxoid fibroma is an uncommon benign cartilaginous tumor of the bone. It occurs most frequently in the metaphysis of long tubular bones, and an epiphyseal location is exceedingly rare. We present here an unusual case of a chondromyxoid fibroma that occurred in the epiphysis of the proximal tibia with an open growth plate. MR imaging findings of this tumor, which has, to the best of our knowledge, never been described in an epiphyseal location, makes the present case unique.

Rare Case of a Chondrosarcoma of the Mandible in a Child

Directory of Open Access Journals (Sweden)

Hendryk Vieweg

2013-01-01

Full Text Available Chondrosarcoma of the mandible is rare, especially in children. The available literature consists mostly of a few case reports which are partly integrated in small studies. Growing this small pool of literature is helpful in solidifying knowledge about this disease and facilitating appropriate treatment for children. Therefore, we present such a case in a 12-year-old boy, exhibit comprehensive and relevant information concerning this entity, and discuss our findings in the context of other publications.

Ectodermal dysplasia-skin fragility syndrome: A rare case report

Directory of Open Access Journals (Sweden)

Subhash Kashyap

2015-01-01

Full Text Available Ectodermal dysplasia/skin fragility syndrome (ED-SFS is a newly described autosomal recessive disorder characterized by skin fragility and blistering, palmoplantar keratoderma, abnormal hair growth, nail dystrophy, and occasionally defective sweating. It results from mutations in the PKP1 gene encoding plakophilin 1 (PKP1, which is an important component of stratifying epithelial desmosomes and a nuclear component of many cell types. Only 12 cases of this rare genodermatosis have been reported so far. We present an unusual case of ED-SFS in a 12-year boy who was normal at birth but subsequently developed skin fragility, hair and nail deformities, abnormal dentition, palmoplantar keratoderma, and abnormal sweating but no systemic abnormality.

A Rare Case of Multifocal Prostatic Blue Nevus

Directory of Open Access Journals (Sweden)

Elias J. Farran

2018-01-01

Full Text Available Prostatic blue nevus is a rare benign pathologic diagnosis most commonly diagnosed incidentally on many different types of prostate specimens. Blue nevus is the deposition of stromal melanin characterized by spindle cells within the fibromuscular stroma which stains positive for melanin-specific stains Fontana-Masson and S100 and stains negative for CD68, HMB45, and iron stains. We report the case of a multifocal and bilateral blue nevus in a 52-year-old Hispanic male who presented with an elevated prostate-specific antigen of 4.3 and mild obstructive lower urinary tract symptoms, found by transrectal ultrasound-guided prostate needle biopsy. The biopsy also revealed benign prostatic tissue with postatrophic hyperplasia and chronic inflammation. This is the 35th reported case of prostatic blue nevus and the third to show multifocal blue nevus.

A rare case of diaphragmatic paralysis due to isolated phrenic nerve ...

African Journals Online (AJOL)

2008-11-04

Nov 4, 2008 ... Isolated phrenic nerve palsy is a rare condition resulting from birth injury, with many possible complications such as diaphragmatic paralysis, pulmonary infection, chronic lung disease, growth failure and even death.1-3 I report a case of neonatal isolated phrenic nerve palsy in an infant delivered.

Transfusion reaction in a case with the rare Bombay blood group

Directory of Open Access Journals (Sweden)

Hayedeh Javadzadeh Shahshahani

2013-01-01

Full Text Available Bombay phenotype is extremely rare in Caucasian with an incidence of 1 in 250,000. When individuals with the Bombay phenotype need blood transfusion, they can receive only autologous blood or blood from another Bombay blood group. Transfusing blood group O red cells to them can cause a fatal hemolytic transfusion reaction. In this study, we report a case with the rare Bombay blood group that was misdiagnosed as the O blood group and developed a hemolytic transfusion reaction. This highlights the importance of both forward and reverse typing in ABO blood grouping and standard cross-matching and performing standard pretransfusion laboratory tests in hospital blood banks.

Foreign body in the nasopharynx- A rare entity: a Case Report

Directory of Open Access Journals (Sweden)

Soumik Saha

2013-06-01

Full Text Available Abstract Nasopharynx is an exceptionally rare anatomical location for foreign body impactation . As an otorhinolaryngologist, one may face challenges in diagnosis and removal of foreign body from airways . A metallic foreign body after being inhaled and ultimately being lodged in the nasopharynx is a rare entity. We report a case of nasopharyngeal foreign body(NFB in a 1½ year old male child. The foreign body was diagnosed by diagnostic nasal endoscopy and documented by X-Ray of nasopharynx - lateral view [FIG.-2] and the same was removed under general anaesthesia. While keeping the child in Rose's position. Keywords foreign body nasopharynx

Borderline personality disorder in men: A literature review and illustrative case vignettes.

Science.gov (United States)

Bayes, Adam; Parker, Gordon

2017-11-01

The aim is to review the salient literature on borderline personality disorder (BPD) in men and link those findings with case vignettes. We provide a literature review and then report case examples of those who met DSM and clinical BPD criteria, and consider the extent to which the small male sub-set corresponded developmentally and phenomenologically with prototypic BPD in women. The review considered phenomenological, epidemiological, biological and developmental BPD factors, finding BPD men evidence elevated substance abuse, and 'externalising' patterns of behavior, antisocial personality traits, violent self-harm and interpersonal aggression, whereas women display more 'internalising' strategies. The five male vignettes enriched the literature review providing support for gender differences reported in our review. The literature and case vignette findings should assist clinicians in recognising that BPD in men may not be as rare as generally viewed, and which may reflect BPD being commonly viewed as weighted to women and being misdiagnosed as an antisocial personality disorder (ASPD) in men. Copyright © 2017 Elsevier B.V. All rights reserved.

Probability estimation of rare extreme events in the case of small samples: Technique and examples of analysis of earthquake catalogs

Science.gov (United States)

Pisarenko, V. F.; Rodkin, M. V.; Rukavishnikova, T. A.

2017-11-01

The most general approach to studying the recurrence law in the area of the rare largest events is associated with the use of limit law theorems of the theory of extreme values. In this paper, we use the Generalized Pareto Distribution (GPD). The unknown GPD parameters are typically determined by the method of maximal likelihood (ML). However, the ML estimation is only optimal for the case of fairly large samples (>200-300), whereas in many practical important cases, there are only dozens of large events. It is shown that in the case of a small number of events, the highest accuracy in the case of using the GPD is provided by the method of quantiles (MQs). In order to illustrate the obtained methodical results, we have formed the compiled data sets characterizing the tails of the distributions for typical subduction zones, regions of intracontinental seismicity, and for the zones of midoceanic (MO) ridges. This approach paves the way for designing a new method for seismic risk assessment. Here, instead of the unstable characteristics—the uppermost possible magnitude M max—it is recommended to use the quantiles of the distribution of random maxima for a future time interval. The results of calculating such quantiles are presented.

Bilateral sertoli-leydig cell tumor of the ovary: A rare case report

Directory of Open Access Journals (Sweden)

Alam Kiran

2009-01-01

Full Text Available Sertoli leydig cell tumors also known as arrhenoblastoma, are a rare member of the sex cord-stromal tumor group of ovarian and testicular cancers, comprising less than 1% of all ovarian tumors, which occur in young adults and are almost always unilateral. We hereby report a case of a 17-year-old female presenting with a short history of irregular menses and an abdominal lump, which was histologically proven to be a bilateral sertoli leydig cell tumor of the ovary, an exceptionally rare entity in itself.

Report of a Rare Case of an Odontogenic Myxoma of the Maxilla ...

African Journals Online (AJOL)

tissue that replaces the cancellous bone and expands the cortex. OMs are locally ... homogenous hypodense lesion of the right side maxilla. Report of a Rare Case of an .... Clinical and radiographic differential diagnosis of OMs may include: ...

Osteoid osteoma of the cervical spine. A case report

International Nuclear Information System (INIS)

Holl, K.; Wurm, G.

2001-01-01

The clinical picture of an OO in the 5th cervical vertebra is illustrated with the case history of a 13 year-old boy. Although in fact a rare case, it becomes evident through reference to and comparison with the literature that this case is a typical one in all aspects. (orig./CB) [de

Congenital insensitivity to pain: Case report of a rare entity

Directory of Open Access Journals (Sweden)

Swati Dahiya

2018-01-01

Full Text Available Hereditary sensory and autonomic neuropathies (HSANs are a group of disorders characterized by insensitivity to noxious stimuli and autonomic dysfunction, associated with pathological abnormalities of the peripheral nerves. Five types of HSAN have been reported in literature, out of which Type V known as congenital insensitivity to pain (CIP is a rare autosomal recessive condition. Self-mutilation is an invariable feature of this disorder, involving the teeth and orofacial structures. This case report describes a case of a 6-year-old girl with CIP brought by her parents for prostheses to replace her self-extracted primary teeth.

Low-grade Myxofibrosarcoma in the Mandible: A Rare Case Report

Directory of Open Access Journals (Sweden)

Neda Kargahi

2017-04-01

Full Text Available Myxofibrosarcoma is a malignant neoplasm of connective tissue origin commonly found in the extremities. It is very rare in the head and neck regions. Only 25 cases of myxofibrosarcoma have been reported in the head and neck regions until 2014. Here we report a 61-year-old male with this neoplasm in the lower border of his mandible. During a two-year follow-up, this case recurred four times despite a complete resection. This study suggested combined surgical and adjuvant radiotherapy for unresectable lesions and tumors with positive margins to prevent recurrence and risk of progression.

Primary peritoneal serous carcinoma: A rare case and palliative approach

Directory of Open Access Journals (Sweden)

Viral M Bhanvadia

2014-01-01

Full Text Available Primary peritoneal serous carcinoma (PPSC is a rare primary malignancy that diffusely involves the peritoneum, indistinguishable clinically and histopathologically from primary serous ovarian carcinoma. The origin of PPSC has not been well characterized. Here we present a case of PPSC diagnosed in ultrasonography-guided fine needle aspiration cytology (FNAC in a 76- old female presenting with ascites, abdominal pain, distension and constipation. PPSC is an unusual tumour but cytomorphology is distinctive enough to diagnose preoperatively. In the case report hereby described PPSC is an inoperable malignancy, hence chemotherapy and palliative care are the only offered treatment.

Solitary Fibrous Tumor of Retromolar Pad; a Rare Challenging Case

Science.gov (United States)

Lotfi, Ali; Mokhtari, Sepideh; Moshref, Mohammad; Shahla, Maryam; Atarbashi Moghadam, Saede

2017-01-01

Solitary fibrous tumor has a wide spectrum of histopathologic features and many tumors show similar microscopic features. This similarity poses diagnostic challenges to the pathologists and immunohistochemical analysis is required in many cases. Moreover, it is a rare entity in orofacial region which consequently would make its diagnosis more challenging in oral cavity. The knowledge of various microscopic patterns of this tumor contributes to a proper diagnosis and prevents unnecessary treatment. This study reports a case of solitary fibrous tumor in the retromolar pad area and discusses its various histological features and differential diagnoses. PMID:28620640

Rare case of primary spinal ependymomatosis occurring in a 26-year-old man: a case report

Directory of Open Access Journals (Sweden)

Kaliaperumal Chandrasekaran

2009-10-01

Full Text Available Abstract Introduction The authors report a rare case of primary spinal ependymomatosis in a young adult man. Multiple primary ependymomatous lesions were seen on magnetic resonance imaging and no anaplasia was identified on the surgical-pathological analysis. The aetio-pathological mechanism and surgical significance of this rare occurrence is discussed. Case presentation A 26-year-old man of Polish origin presented with a ten-day history of pain in the left leg and lower back. This was followed by difficulty in urinating and a decrease in sensation in both legs. Examination revealed pyramidal signs and mild weakness in both lower limbs. He had early sphincter involvement requiring catheterization. Magnetic resonance imaging of the brain was normal. However, that of the spinal cord revealed multiple intradural spinal lesions, both intra- and extramedullary, extending from the cervical cord down to the cauda equina roots. T12-L1 laminectomy was performed. Multiple intradural, extra- and intra-medullary tumors were seen. After the operation, the patient deteriorated with a sensory level at T4. Post-operative cranio-spinal radiotherapy was administered but there was no clinical improvement in the lower limbs. Conclusion Primary spinal ependymomatosis is a rare phenomenon involving multiple spinal segments in the absence of a primary intracranial tumor. Radical excision is unrealistic in this condition. Biopsy followed by radiotherapy is the preferred method of treatment.

Rare case of pure medial subtalar dislocation in a basketball player ...

African Journals Online (AJOL)

Optimal management of subtalar dislocations is immediate closed reduction with procedural sedation. We report a very rare case of a closed subtalar dislocation without any related fractures. A 22 year old male was admitted to the emergency department with pain and ankle deformity following an inversion injury during a ...

Vaginal Endosalpingiosis Case Report: A Rare Entity Presenting as Intermenstrual Bleeding

Directory of Open Access Journals (Sweden)

Sara Câmara

2017-01-01

Full Text Available Endosalpingiosis is a benign and rare entity whose pathophysiology remains unknown. It has been described in pelvic organs, the abdomen, or axillar lymph nodes. Its underrecognition has occasionally led to its misinterpretation for an adenocarcinoma. This case reports the treatment and follow-up of vaginal endosalpingiosis, presenting as a vaginal polyp in a premenopausal women with intermenstrual bleeding. To our knowledge this is the first reported case of vaginal endosalpingiosis and the second mucosal localization after bladder endosalpingiosis.

A rare case of short stature: Say Meyer syndrome

Directory of Open Access Journals (Sweden)

T S Karthik

2013-01-01

Full Text Available Introduction: Say Meyer syndrome is rare X linked condition characterized by developmental delay, short stature and metopic suture synostosis. We are reporting a case of Say Meyer syndrome presented to our hospital for short stature and developmental delay at age 3½ years. Case Report: A 3½-year-old boy presented to our hospital for decreased growth velocity from the age of 1 year. History revealed the boy had a birth weight of 2.3 kg, had an episode of seizures in the neonatal period. He was born to non-consanguineous marriage. He had global developmental delay and there was a lack of bowel and bladder control. History did not reveal any hearing or visual impairment. No history of any chronic systemic illnesses. Magnetic resonance imaging (MRI brain revealed mild diffuse frontotemporal atrophy with multiple irregular gliotic areas in bilateral frontal lobes. Diffuse white matter volume loss in bilateral cerebral hemispheres. Diffuse thinning of corpus callosum. Diffuse periventricular hyper intensity on T2W and fluid attenuated inversion recovery sequences. Conclusion: Say Meyer syndrome is rare X linked condition characterized by developmental delay, short stature and metopic suture synostosis. Characteristic MRI brain findings include diffuse frontotemporal atrophy with multiple gliotic areas in frontal lobes. Diffuse white matter volume loss in bilateral cerebral hemispheres.

Approaches to Feminist Therapy: A Case Study Illustration

Directory of Open Access Journals (Sweden)

Selvira Draganović

2011-01-01

Full Text Available This paper deals with the emergence and development of feminism in general and feminist psychology with special emphasis on feminist's reaction to traditional assumptions in Freudian psychology and male dominated theory and research. Feminist movement simply started as a women's activism which later claimed political identity and finally ended as women's liberation movement. Feminists simply advocate gender equality. Emergence of feminist psychology movement and appearance of significant female figures in psychology is further explained along with their contribution to the theory, personality and specific developmental issues along with its input to the therapy and counseling field. The influence of four feminist philosophical approaches namely, liberal feminism, cultural feminism, radical feminism and social feminism are also shortly discussed alongside with their practical implications. Above and beyond, feminist contribution to the therapy field reflected in offering specific therapy goals are also discussed. Self esteem as a core issue and one of feminist therapy goals is finally discussed and presented through a specific case study illustration. Feminist therapy is important therapy approach with significant therapy goals contribution in relation to women's mental health issues. Consideration of sex, gender, cultural diversity, etiology, diagnosis and treatment is feminist request for successful therapy because fixing woman for functioning in a dysfunctional society seems not enough.

Corneal ulcer due to a rare coelomycetes fungus Chaetomium strumarium: Case report and global review of Chaetomium keratomycosis

Directory of Open Access Journals (Sweden)

Mamatha Reddy

2017-01-01

Full Text Available We present a rare case of corneal ulcer caused by a species of a coelomycetes fungus, Chaetomium strumarium. This fungal genus is a rare causative agent of keratomycosis, with only a handful of cases reported. The clinical presentation, investigative techniques, and preliminary management of our patient are reported. The cases reported in global literature are also summarized in a tabular form in the discussion.

Corneal ulcer due to a rare coelomycetes fungus Chaetomium strumarium: Case report and global review of Chaetomium keratomycosis.

Science.gov (United States)

Reddy, Mamatha; Venugopal, Ramya; Prakash, Peralam Yegneswaran; Kamath, Yogish Subraya

2017-09-01

We present a rare case of corneal ulcer caused by a species of a coelomycetes fungus, Chaetomium strumarium. This fungal genus is a rare causative agent of keratomycosis, with only a handful of cases reported. The clinical presentation, investigative techniques, and preliminary management of our patient are reported. The cases reported in global literature are also summarized in a tabular form in the discussion.

Beautiful Surfaces. Style and Substance in Florentius Schuyl's Illustrations for Descartes' Treatise on Man.

Science.gov (United States)

Chan, Eleanor

2016-01-01

The assumption that the Cartesian bête-machine is the invention of René Descartes (1596-1650) is rarely contested. Close examination of Descartes' texts proves that this is a concept founded not on the basis of his own writings, but a subsequent critical interpretation, which developed and began to dominate his work after his death. Descartes' Treatise on Man, published posthumously in two rival editions, Florentius Schuyl's Latin translation De Homine (1662), and Claude Clerselier's Traité de l'homme, has proved particularly problematic. The surviving manuscript copies of the Treatise on Man left no illustrations, leaving both editors the daunting task of producing a set of images to accompany and clarify the fragmented text. In this intriguing case, the images can be seen to have spoken louder than the text which they illustrated. This paper assesses Schuyl's choice to represent Descartes' Man in a highly stylized manner, without superimposing Clerselier's intentions onto De Homine.

A rare disease in an atypical location - Kimura's Disease of the upper extremity

Energy Technology Data Exchange (ETDEWEB)

Lam, Alan Cheuk Si; Lau, Vince Wing Hang [Queen Mary Hospital, Department of Radiology, Hong Kong (China); Au Yeung, Rex Kwok Him [University of Hong Kong, Department of Pathology, Li Ka Shing Faculty of Medicine, Hong Kong (China)

2015-12-15

Kimura's disease is a rare chronic inflammatory disorder predominantly affecting young Asian male patients, occurring mainly in the head and neck regions. Kimura's disease of the upper extremity is extremely rare, and previous case reports in the literature show similar imaging characteristics with consistent location at the medial epitrochlear region, predominantly with unilateral involvement. We present the first reported case of Kimura's disease affecting the anterolateral aspect of the upper arm, sparing the medial epitrochlear region, illustrating that with typical MR appearance and serology, the involvement of this rare disease in an atypical location still warrants consideration of this diagnosis. There was also bilateral asymmetrical involvement in our patient, suggesting the possibility of a propensity for Kimura's disease affecting the upper extremities to have bilateral involvement, which may necessitate imaging of the clinically asymptomatic contralateral limb in these patients for early lesion identification and treatment. (orig.)

A rare disease in an atypical location - Kimura's Disease of the upper extremity

International Nuclear Information System (INIS)

Lam, Alan Cheuk Si; Lau, Vince Wing Hang; Au Yeung, Rex Kwok Him

2015-01-01

Kimura's disease is a rare chronic inflammatory disorder predominantly affecting young Asian male patients, occurring mainly in the head and neck regions. Kimura's disease of the upper extremity is extremely rare, and previous case reports in the literature show similar imaging characteristics with consistent location at the medial epitrochlear region, predominantly with unilateral involvement. We present the first reported case of Kimura's disease affecting the anterolateral aspect of the upper arm, sparing the medial epitrochlear region, illustrating that with typical MR appearance and serology, the involvement of this rare disease in an atypical location still warrants consideration of this diagnosis. There was also bilateral asymmetrical involvement in our patient, suggesting the possibility of a propensity for Kimura's disease affecting the upper extremities to have bilateral involvement, which may necessitate imaging of the clinically asymptomatic contralateral limb in these patients for early lesion identification and treatment. (orig.)

PRIMARY GIANT HYDATID DISEASE OF THE SPLEEN: A RARE CASE REPORT WITH REVIEW OF LITERATURE

Directory of Open Access Journals (Sweden)

Subramanyam

2015-02-01

Full Text Available The most common organ involved in hydatid disease is the liver, followed by the lungs. Hydatid disease of spleen is a rare clinical condition, as even in the endemic region the frequency is reported to be 0.5 – 4% of abdominal hydatid diseases. Most commonly splenic involvement is secondary i.e., along with other organs. Primary hydatid diseases in s pleen is rare, here we are reporting a rare case of primary splenic hydatid disease

Diagnosis and treatment of talar dislocation fractures illustrated by 3 case reports and review of literature

NARCIS (Netherlands)

Haverkort, J. J M; Leenen, L. P H; van Wessem, K. J. P.

2015-01-01

Introduction Talar fractures are a rare type of fractures (less than 1%). They are difficult to treat and outcome is often complicated by arthritis and avascular necrosis. In this article three cases are presented with different types of dislocated talar neck fractures. Anatomy of the talus,

Primary Peritoneal Hydatid Cyst Presenting as Ovarian Cyst Torsion: A Rare Case Report

OpenAIRE

Gandhiraman, Kavitha; Balakrishnan, Renukadevi; Ramamoorthy, Rathna; Rajeshwari, Raja

2015-01-01

Hydatid cyst disease is a zoonotic disease caused by Echinococcus granulosus, E.multilocularis or E.Vogli. The most common primary site is liver (75%) followed by lungs (5-15%) and other organs constitute 10-20%. Peritoneal hydatid cysts are very rare especially primary peritoneal hydatid. Secondary peritoneal hydatid cysts are relatively common, which usually occurs due to rupture of primary hepatic hydatid cyst. We present a rare case of large primary peritoneal hydatid cyst misdiagnosed as...

Humeral Metastasis from Cervical Cancer: A Rare Case Report

OpenAIRE

Sonia Chhabra; KanikaTaneja; Megha Ralli; Sunita Singh; Aditi Arora; Sohrab Arora; Pansi Gupta

2015-01-01

Long bone metastasis in cervical cancer is a rare presentation generally seen in the lumbar column or ribs. The reported rates of bone metastases are between 15%-29%. It is associated with poor prognosis. Bone scan and magnetic resonance imaging are useful techniques for diagnosis. In this case report, a 32-year old female with a previous history of cervical carcinoma FIGO stage IIIA presented with severe pain and swelling in her right humerus. X-ray and magnetic resonance imag...

A rare case of breast carcinoma co-existing with axillary mantle cell lymphoma

Directory of Open Access Journals (Sweden)

Scally John

2003-12-01

Full Text Available Abstract Background Mantle cell lymphoma (MCL is a rare variety of non-Hodgkin's lymphoma which originates from CD5+ B-cell population in the mantle zones of lymphoid follicles. Coexistence of such tumours in the axillary lymph nodes with invasive breast cancers without prior history of adjuvant chemotherapy or radiotherapy has not been previously reported in literature. Case report We report a rare case of breast cancer co-existing with stage I mantle cell lymphoma of the ipsilateral axillary lymph node detected fortuitously by population screening. Conclusion Though some studies have tried to prove breast carcinomas and lymphomas to share a common molecular or viral link, more research needs to be done to establish whether such a link truly exists.

Mixed Pyolaryngocele: A Rare Case of Deep Neck Infection

Directory of Open Access Journals (Sweden)

Rachid Mahdoufi

2017-07-01

Full Text Available Introduction: Pyolaryngocele is a very rare and serious complication of laryngocele. It can present as deep neck space infection and mislead the diagnosis. Our aim is to bring this unusual entity to the attention of surgeons and describe its clinical features. Case Report: We report a case of a 45-year-old male patient with a five-week history of neck swelling, dysphonia, dyspnea and odynophagia. An urgent CT scan showed a mixed pyolaryngocele. The management consisted of a high dose antibiotic and an excision of the residual laryngocele via an external approach. Conclusion: A pyolaryngocele is an unusual complication of laryngocele, which becomes secondarily infected, causing many symptoms. Removing the laryngocele is still the best treatment option to prevent this complication and recurrence.

A rare case of small bowel volvulus after jenjunoileal bariatric bypass requiring emergency surgery: a case report

Directory of Open Access Journals (Sweden)

Patel Pranav H

2012-03-01

Full Text Available Abstract Introduction Bariatric surgery is on the increase throughout the world. Jejunoileal bypass bariatric procedures have fallen out of favor in western surgical centers due to the high rate of associated complications. They are, however, performed routinely in other centers and as a consequence of health tourism, management of complications related to these procedures may still be encountered. Case presentation We describe a rare case of small bowel obstruction in a 45-year-old British Caucasian woman, secondary to a volvulus of the jejunoileal anastomosis following bariatric bypass surgery. The pre-operative diagnosis was confirmed by radiology. We describe a successful surgical technique for this rare complication. Conclusions Bariatric surgery may be complicated by bowel obstruction. Early imaging is vital for diagnosis and effective management. The use of our surgical technique provides a simple and effective approach for the successful management of this bariatric complication.

Hypokalaemic Periodic Paralysis in a Patient with Subclinical Hyperthyroidism: A Rare Case

OpenAIRE

Hegde, Swati; Shaikh, Mohammed Aslam; Gummadi, Thejaswi

2016-01-01

Thyrotoxic Periodic Paralysis (TPP) is an uncommon disorder. Though many cases of hypokalaemic periodic paralysis are reported in overt hyperthyroidism, hypokalaemic paralysis in subclinical hyperthyroidism is very rare. Subclinical hyperthyroidism is characterised by circulating TSH levels below reference range and normal thyroid hormone levels. We describe a case of 32-year-old Asian male who presented to the emergency department with acute onset weakness and hypokalaemia with no previous h...

Case Report

DEFF Research Database (Denmark)

Bilgin-Freiert, Arzu; Fugleholm, Kåre; Poulsgaard, Lars

2015-01-01

We report a case of an intraneural ganglion cyst of the hypoglossal canal. The patient presented with unilateral hypoglossal nerve palsy, and magnetic resonance imaging showed a small lesion in the hypoglossal canal with no contrast enhancement and high signal on T2-weighted imaging. The lesion...... irradiation as an option. This case illustrates a very rare location of an intraneural ganglion cyst in the hypoglossal nerve. To our knowledge there are no previous reports of an intraneural ganglion cyst confined to the hypoglossal canal....

A rare case of acute intermittent porphyria with ichthyosis vulgaris in a young boy

Directory of Open Access Journals (Sweden)

Garima Agrawal Varshney

2018-01-01

Full Text Available Acute intermittent porphyria (AIP and ichthyosis vulgaris both are autosomal dominant disorders with incomplete penetrance caused by the deficiency of porphobilinogen deaminase enzyme and filaggrin protein, respectively. We report a rare case of a 9-year-old boy having two genetic diseases with an unclear association. An acute attack of AIP is characterized by gastrointestinal symptoms and neuropsychiatric manifestations. Although rare in the first decade of life, the presence of reddish urine with a typical presentation such as abdominal pain, hypertension, seizure, and paresthesias lead us to the diagnosis of AIP. The precipitating factor in the present case was prolonged fasting in Ramadan.

Rare earths and actinides

International Nuclear Information System (INIS)

Coqblin, B.

1982-01-01

This paper reviews the different properties of rare-earths and actinides, either as pure metals or as in alloys or compounds. Three different cases are considered: (i) First, in the case of 'normal' rare-earths which are characterized by a valence of 3, we discuss essentially the magnetic ordering, the coexistence between superconductivity and magnetism and the properties of amorphous rare-earth systems. (ii) Second, in the case of 'anomalous' rare-earths, we distinguish between either 'intermediate-valence' systems or 'Kondo' systems. Special emphasis is given to the problems of the 'Kondo lattice' (for compounds such as CeAl 2 ,CeAl 3 or CeB 6 ) or the 'Anderson lattice' (for compounds such as TmSe). The problem of neutron diffraction in these systems is also discussed. (iii) Third, in the case of actinides, we can separate between the d-f hybridized and almost magnetic metals at the beginning of the series and the rare-earth like the metals after americium. (orig.)

A Rare Case of Neonatal Hypophosphatasia: A Case Report

Directory of Open Access Journals (Sweden)

Nasim Pouralizadeh

2018-03-01

Full Text Available Hypophosphatasia is a rare hereditary disorder of bone metabolism.In this article, we presented the case of a male infant with a soft skull and short, deformed limbs at birth, followed by seizures and respiratory distress during admission in the neonatal intensive care unit (NICU. Prenatal ultrasound showed limb hypoplasia, skull hypomineralization, and polyhydramnios. Seizures occurred on day nine of admission. The neonate was intubated after pneumonia on day 12of birth and died due to the same cause and respiratory failure on day 14. Clinical presentation and low alkaline phosphatase (ALP confirmed the diagnosis of hypophosphatasia. The disorder covers a spectrum of severe neonatal type with severe hypomineralization to various adult types with osteomalacia and dental problems. Prenatal hypophosphatasia is diagnosed based on the clinical signs, including soft skull, short limbs, breathing difficulty, seizures, respiratory distress, laboratory results (low ALP and high pyridoxal 5-phosphate, and radiographic findings (hypomineralization and metaphyseal dysplasia.

Juvenile Idiopathic Arthritis Onset in a Neonate: A Rare Case Report

Directory of Open Access Journals (Sweden)

Abdolreza Malek

2017-06-01

Full Text Available Background: A common type of chronic arthritis in children and adolescents is juvenile idiopathic arthritis (JIA.According to the International League of Associations for Rheumatology (ILAR classification, JIA diagnostic criteria include age under 16 years and disease duration of six-weeks. Based on the number of involved joints in the first sixmonths of disease onset, JIA is categorized into oligoarticular or polyarticular subtypes. Age is a characteristic factor in the diagnosis of disease subsets; it is worth mentioning that cases younger than six months of age are seldom found in any of the subtypes. Case report: In this report, we present a rare case of JIA in an infant, presenting at 20 days of age. Effusion of the right hip joint was one of the primary manifestations of the disease. During hospitalization, she went through sepsis workup and a four-week antibiotic therapy for management of lower limb pseudoparalysis. In spite of antibiotic therapy, she developed effusion of a second joint. According to the course and duration of symptoms and ILAR classification forJIA, oligoarticular JIA was diagnosed and treated.Conclusion: In this case, infectious diseases, such as tuberculosis and brucellosis, and malignancies were ruled out as a cause of inflammation through bone marrow aspiration, culture, and tests; ultrasound and magnetic resonance imaging showed no lytic and sclerotic lesions or a fracture. Our experience showed a rare case of JIA and suggested that JIA must be considered in children with joint inflammation at any age

A Rare Case of Pott's Disease (Spinal Tuberculosis) Mimicking Metastatic Disease in the Southern Region of Denmark.

Science.gov (United States)

Osmanagic, Azra; Emamifar, Amir; Christian Bang, Jacob; Jensen Hansen, Inger Marie

2016-06-07

Pott's disease (PD) or spinal tuberculosis is a rare condition which accounts for less than 1% of total tuberculosis (TB) cases. The incidence of PD has recently increased in Europe and the United States, mainly due to immigration; however, it is still a rare diagnosis in Scandinavian countries, and if overlooked it might lead to significant neurologic complications. A 78-year-old woman, originally from Eastern Europe, presented to the emergency department with a complaint of nausea, vomiting, weight loss, and severe back pain. On admission she was febrile and had leukocytosis and increased C-reactive protein. Initial spinal x-ray was performed and revealed osteolytic changes in the vertebral body of T11 and T12. Magnetic resonance imaging (MRI) of the spine illustrated spondylitis of T10, T11, and T12, with multiple paravertebral and epidural abscesses, which was suggestive of PD. Polymerase chain reaction (PCR) of the patient's gastric fluid was positive for Mycobacterium tuberculosis (MT). Based on MRI and PCR findings, standard treatment for TB was initiated. Results of the spine biopsy and culture showed colonies of MT and confirmed the diagnosis afterwards. Due to the instability of the spine and severe and continuous pain, spine-stabilizing surgery was performed. Her TB was cured after nine months of treatment. PD is an important differential diagnosis of malignancy that should be diagnosed instantly. History of exposure to TB and classic radiologic finding can help make the diagnosis.

A case report of gastric lymphocytic phlebitis, a rare mimic for malignancy

Directory of Open Access Journals (Sweden)

Daniel L. Chan

Full Text Available Introduction: Lymphocytic phlebitis is a benign condition characterised by inflammation of the veins and rarely affects the gastrointestinal tract. Reported cases present as acute abdomen and involve the colon or small intestine. We report the fourth case of gastric lymphocytic phlebitis in the literature. Presentation of case: A 74-year-old female presented with eight weeks of abdominal pain. Findings at endoscopy were suggestive of a malignant ulcer on the greater curvature of antrum, while biopsies showed chronic gastritis without malignancy. Appearance at diagnostic laparoscopy was consistent with a malignant gastric ulcer with serosal changes. Due to persistent pain and the macroscopic appearance, she proceeded to have an open subtotal gastrectomy and D2 lymph node clearance. Despite macroscopic appearance, the microscopic examination demonstrated no malignancy, and was consistent with lymphocytic phlebitis with overlying ulceration. Discussion: This case was a mimic for gastric malignancy, with the benign diagnosis only being made after surgical resection. Gastric lymphocytic phlebitis is a rare differential diagnosis for gastric ulcers when biopsies are negative, although preoperative diagnosis is difficult given the lesions do not involve the mucosa. Conclusion: If clinical history and endoscopic findings are suspicious for malignancy, despite normal biopsies, an aggressive surgical resection remains reasonable given the rarity gastric lymphocytic phlebitis. Keywords: Lymphocytic phlebitis, Gastrectomy, Gastric vasculitis, Gastric ulcer, Case report

Nodulocystic basal cell carcinoma arising directly from a seborrheic keratosis: A rare case report

Directory of Open Access Journals (Sweden)

Kana Shibao

2016-01-01

Full Text Available Seborrheic keratoses (SKs are common epidermal tumors composed of benign keratinocytes. Malignant skin tumors including basal cell carcinoma (BCC rarely arise within SKs. We report a rare case of an 82-year-old man with nodulocystic BCC that appeared at the center of a scaly hyperpigmented SK that had been presented for more than 10 years. It was histologically confirmed that CK19-positive BCC arose directly from the wall of the pseudohorn cyst, a part of the SK. Nodular and/or cystic BCC also rarely arise within SKs while the most common histologic type of BCC within SKs is the superficial type. Careful observation of SKs is important even though it is rarely a background condition for malignant transformation.

Colloidon baby – Rare case with preventable complications

Directory of Open Access Journals (Sweden)

Janardhan Bommakanti

2015-01-01

Full Text Available Colloidon baby is a rare congenital disorder characterized clinical-ly by parchment like taught membrane covering the whole body at the time of birth, which subsequently develops Non bullous ichthyosiform erythroderma or Lamellar ichthyiosis in most cases and in few cases other ichthyosiform disorders. The colloidon membrane spontaneously desquamates within 2 weeks or up to 3 months in few cases. Herein, we present 2 cases of colloidon babies born to consanguineously married couples of which the first baby was born at term by normal vaginal delivery and second baby born prematurely by caesarean section. Both 1st & 2nd baby were delivered in different private hospitals in villages of Nizamabad district, Telangana state and reported to tertiary level children’s hospital in Hyderabad city on 4nd and 6th day of life respectively with complaints of colloidon membrane and macera-tion of skin in diaper area, was admitted in Neonatal intensive care unit (NICU in humidified incubator, treated with emollients, intra-venous fluids and prophylactic antibiotics to avoid complications. Nursing care is of prime importance. This presentation was aimed at stressing not only the importance of early recognition by pedia-trician & timely referral to dermatologist and ophthalmologist for saving life of affected baby but also equal importance to proper nursing care.

A Rare Case Of Graves’ Disease With Splenomegaly And Pancytopenia

Directory of Open Access Journals (Sweden)

Elayne Christinne Marcelino e Silva

2017-07-01

Full Text Available Introduction: Splenomegaly and pancytopenia are rare complications of Graves' disease with few reports in the literature about this association. The pathogenesis is unknown and immunological mechanisms seem to be involved. The possibility of hyperthyroidsm should always considered in patients with pancytopenia. Objective: Describe  clinical case of association between Grave's disease, splenomegaly and pancytopenia. Method: This is a case report, obtained through data from medical records of a reference hospital located in the city of Juazeiro do Norte, Ceara, Brazil. Case report: Patient, 46 years old, female, sought treatment at a reference hospital with abdominal pain that started two days earlier, prevalent in mesogastric region and left hypochondrium very intense and recurrent, associated with significant consuptive syndrome ( loss of 10 Kg in 4 months, asthenia, dyspnea on minimum exertion, irritability and fine tremor in extremitie. SHe denied fever, palpitations, heat intolerance, skin ou eye changes. A diffuse thyroid enlargement with the presence of thrill and murmur, digital clubbing, fixed and bright look, light exophthalmos and splenomegaly about 6 cm below the left costal margin were abserved after physical examination. Ultrasound examination (USG of the abdomen and CT scan showed moderate splenomegaly. Laboratory tests showed normocytic and normochromic anemia, leukocytosis and mild thrombocytopenia. Thyroid USG showed characteristic features of Graves' disease, a bone marrow biopsy revealed maturation preserved in all strains and lack of fibrosis and megakaryocytes present in normal number without atypia. treatment was set with propylthiouracil 300 mg a day and after the first revaluation after hospital discharge three weeks later a regression of splenomegaly has been observed. Conclusion: This case ilustrates the rare association between hyperthyroidism and splenomegaly with pancytopenia.

A rare case of synovial sarcoma of the prostate | Dhabalia | African ...

African Journals Online (AJOL)

Prostatic synovial sarcomas are exceedingly rare. To our knowledge, only six primary cases have been reported so far. We herein describe a primary synovial sarcoma of the prostate seen in a 25- year-old male patient, the youngest patient seen with this disease to date. He was referred to our department with the diagnosis ...

Chylous ascites and lymphangiectasia in focal segmental glomerulosclerosis--a rare coexistence: a case report.

Science.gov (United States)

Lahiri, Durjoy; Agarwal, Rakesh; Roy, Manoj Kumar; Biswas, Amrita

2015-02-09

Nephrotic syndrome is considered a rare cause of chylous ascites. Intestinal lymphangiectasia in a background of chylous ascites and without any lymphatic obstruction has been reported in association with yellow nail syndrome, which is a rare clinical occurrence in itself. The existence of chylous ascites, duodenal and splenic lymphangiectasia (without any lymphatic obstruction) and nephrotic syndrome in the form of focal segmental glomerulosclerosis in the same patient makes this case the first of its kind to be reported in the literature. Here we report the case of a 54-year-old Asian man who presented with recurrent episodes of anasarca for approximately 25 years. He was subsequently found to have chylous ascites, lymphangiectasia and persistent proteinuria. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified. A lymphangiogram, which was performed with the purpose of addressing the intestinal lymphangiectasia, failed to demonstrate any abnormality of lymphatic channels. He was put on oral steroids with consequent remission of his oedema and proteinuria. This case highlights the fact that duodenal and splenic lymphangiectasia can exist in a scenario of chylous ascites without any obvious obstruction of lymphatic channels and in the absence of yellow nail syndrome. This case also signifies that chylous ascites may be a rare presenting feature of nephrotic syndrome and hence this aspect should be considered while in diagnostic dilemma regarding such a clinical presentation.

Intraosseous Polymorphous Low Grade Adenocarcinoma of the Mandible: Report of a Rare Case

Directory of Open Access Journals (Sweden)

SM R Prakash

2010-01-01

Full Text Available Polymorphous low-grade adenocarcinoma (PLGA is an entity under the subclassification of adenocarcinoma from salivary glands. PLGA is an unusual tumor that occur: in the minor salivary gland, particularity in the palate followed by buccal mucosa, upper lip, retromolar area and base of the tongue- The occurance of PLGA in locations other than these is exceedingly rare. We report a rare case of PLGA occuring in the anterior mandible. The clinical, radiographic, computed tomographic and histopathologic findings are discussed.

Pyknodysostosis: report of a rare case with review of literature

OpenAIRE

Ramaiah, Kiran Kumar Kotagudda; George, Giju Baby; Padiyath, Sheeba; Sethuraman, Rupak; Cherian, Babu

2011-01-01

Pyknodysostosis is a rare autosomal recessive disorder characterized by the post natal onset of short limbs, short stature, and generalized hyperostosis along with acro-osteolysis with sclerosis of the terminal phalanges, a feature that is considered essentially pathognomonic. Other features include persistence of fontanelles, delayed closure of sutures, wormian bones, absence of frontal sinuses, and obtuse mandibular gonial angle with relative mandibular prognathism. We report a case of 17-y...

An overview of exposure to ethanol-containing substances and ethanol intoxication in children based on three illustrated cases

Directory of Open Access Journals (Sweden)

Kam Lun Hon

2018-01-01

Full Text Available Alcohol addiction and intoxication are major health problems worldwide. Acute alcohol intoxication is well reported in adults and adolescents but less frequently reported in children of younger ages. We report three anonymized cases of pediatric ethanol exposure and illustrate the different mechanisms of intoxication. In all cases, a focused history is the key to prompt diagnosis and timely management. Physicians should be aware of this potential poison in children presented with acute confusional or encephalopathic state. In contrast, neonates with ethanol intoxication may present with nonspecific gastrointestinal symptomatology. Urgent exclusion of sepsis, electrolyte imbalance, drug intoxication, and surgical abdominal condition is critical. Using these illustrated cases, we performed a narrative literature review on issues of exposure to ethanol-containing substances and ethanol intoxication in children. In conclusion, a high level of suspicion and interrogation on ethanol or substance use are essential particularly in the lactating mother for an accurate and timely diagnosis of ethanol intoxication to be made.

A rare case of primary amenorrhea

Directory of Open Access Journals (Sweden)

Himadri Bal

2016-01-01

Full Text Available Crytomenorrhea arises usually due to the imperforate hymen, sometimes due to vaginal septum, rarely due to cervical agenesis. Here, we present a case of primary amenorrhea resulting from cervical agenesis in a 38-year-old woman. She presented with primary amenorrhea and cyclic lower abdominal pain. She had undergone some surgery 5 years back, details of which were not available. On examination secondary sexual characters were normal, per abdomen there was an 18 weeks size firm abdominopelvic mass. Local vaginal examination showed a blind vaginal pouch. A clinical diagnosis of hematometra due to transverse vaginal septum was made. However, magnetic resonance imaging pelvis suggested hematometra with cervical stenosis. The patient was taken up for examination under anaesthesia (EUA and exploratory laparotomy. On opening the abdomen uterus found to be enlarged with dense adhesions all around and signs of endometriosis. Extensive adhesiolysis revealed bilateral chocolate cysts of ovaries with hematosalpinges and peritubal adhesions. Hysterotomy and drainage of tarry contents were followed by an exploration of the uterine cavity. The lower pole ended blindly with no evidence of any cervix. Peroperative diagnosis of cervical agenesis leading to hematometra and endometriosis was made. A subtotal hysterectomy with bilateral salpingo-oophorectomy was done. Histopathological examination confirmed ovarian endometriosis and adenomyosis of uterus. Though reconstructive surgery for cervical dysgenesis has been successful in some cases, hysterectomy is generally recommended for cervical agenesis.

Angiomyomatous hamartoma: a rare case report with review of the literature

Directory of Open Access Journals (Sweden)

Naseem Ansari

2009-12-01

Full Text Available Normal 0 false false false EN-GB X-NONE X-NONE MicrosoftInternetExplorer4 Angiomyomatous hamartoma of the lymph node is a benign vascular disorder of unknown aetiology, first described in 1991. However, to the best of our knowledge, it still remains a rare entity with only 17 cases reported so far. We report a case in an 82 year old male, present a review of literature, and stress the importance of diagnosis of this benign condition which obviates the need for unnecessary and extensive surgical resection.

Clinical and radiological spectrum of cleidocranial dysplasia: A rare case report

Directory of Open Access Journals (Sweden)

Sneha H Choudhary

2017-01-01

Full Text Available Cleidocranial dysplasia (CCD is a rare congenital disorder which affects the bones and teeth. CCD usually has an autosomal dominant inheritance pattern, however, it may appear spontaneously in some cases. The most common features seen in CCD are aplastic or hypoplastic clavicles, late closure of fontanelle, open skull sutures, delayed exfoliation of deciduous teeth, delayed or failed eruption of permanent teeth, and supernumerary teeth. Here, we report a case of CCD in a 17-year-old female without any family history who presented with the complaint of missing teeth.

A rare case of complete second arch branchial fistula in a 7-year-old child

OpenAIRE

Shankar, Venkateswara Gomathi; Babu, Thirunavukkarasu Arun; Swami, Hartimath Basavanand

2012-01-01

Branchial fistulae are formed due to the abnormal persistence of the embryonic branchial clefts. Complete branchial fistula with internal and external opening is extremely rare. We report a rare case of complete second arch branchial fistulae in a 7-year-old boy, which was confirmed by a fistulogram. The tract was completely excised and the patient was successfully treated.

A rare case of complete second arch branchial fistula in a 7-year-old child.

Science.gov (United States)

Shankar, Venkateswara Gomathi; Babu, Thirunavukkarasu Arun; Swami, Hartimath Basavanand

2012-07-01

Branchial fistulae are formed due to the abnormal persistence of the embryonic branchial clefts. Complete branchial fistula with internal and external opening is extremely rare. We report a rare case of complete second arch branchial fistulae in a 7-year-old boy, which was confirmed by a fistulogram. The tract was completely excised and the patient was successfully treated.

Illustrating the Interaction of Nature and People in Ecosystem Services: The Case of Terroir in Wine

Science.gov (United States)

Nicholas, K. A.

2014-12-01

The ecosystem services (ES) approach is increasingly used in research and policy, with the Common International Framework on Ecosystem Services (CICES) "cascade" gaining traction as a framework for conceptualizing the production of ecosystem services by the natural environment, and then people consuming these services and obtaining benefits depending on their values. However, uptake of the ES concept on the ground by ecosystem managers, and understanding by everyday citizens, is still limited. One barrier is the challenge of providing tangible, examples of everyday benefits and values that people can readily connect with the biophysical structures and functions that underlie their provision. Winegrowing offers one promising case to illustrate the linkages all along the chain of production and consumption of ecosystem services. The sensitive winegrape has long been known for its properties of terroir, where the taste of wine reflects the environmental conditions of the place where it is grown, a feature valued by consumers. Here the CICES framework is illustrated with the case of winegrowing, demonstrating that the current linear model of natural production and human consumption of ES needs to be modified for this case because people influence each of the five stages by shaping and responding to their environment, producing a two-way interaction between people and the environment throughout. For example, while natural drivers such as climate and soils are key to the provision of the service of winegrape yields, landowners modify the biophysical environment through site selection and growers modify plant ecophysiological function through farming practices such as pruning and irrigation in order to influence the final service. Similarly, winemakers' expertise is needed to transform the service of winegrape yields into the product of wine that can be enjoyed and valued by consumers, whose preferences shape wine styles as well. This case illustrates how incorporating

Prune Belly syndrome: A rare case report.

Science.gov (United States)

Samal, Sunil Kumar; Rathod, Setu

2015-01-01

Prune Belly syndrome (PBS) is a rare congenital anomaly of uncertain etiology almost exclusive to males. We report a case of term male baby born to a 39-year-old grand multipara with previous four normal vaginal births. There was no history of genetic or congenital anomaly in her family. Examination of the baby revealed hypotonia, deficient abdominal muscle, cryptorchidism, palpable kidney, and bladder. Ultrasound examination of the abdomen revealed bilateral gross hydronephrosis and megaureter. Provisional diagnosis of PBS was made and the baby was admitted in neonatal intensive care units for further management. Routine antenatal care with ultrasonography will help in detecting renal anomalies, which can be followed postnatally. Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course.

A rare case of 46,XX gonadal dysgenesis and Mayer-Rokitansky-Kuster-Hauser syndrome.

Science.gov (United States)

Manne, Sriharibabu; Veeraabhinav, C H; Jetti, Mounica; Himabindu, Yalamanchali; Donthu, Kiranmai; Badireddy, Mutyalarayudu

2016-01-01

46,XX gonadal dysgenesis is a rare genetically heterogeneous disorder characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of secondary sexual characteristics and a normal 46,XX karyotype. The phenotypic manifestations of MRKH syndrome may sometimes overlap with various other syndromes and require accurate delineation. The coexistence of both these disorders is extremely rare. Here, we report a case of 46,XX gonadal dysgenesis and MRKH syndrome with anatomically dispersed congenital anomalies unique among reported cases.

Nevus of Ota with Rare Palatal Involvement: A Case Report with Emphasis on Differential Diagnosis

Directory of Open Access Journals (Sweden)

Gaurav Sharma

2011-01-01

Full Text Available Nevus of Ota, a dermal melanocytic nevus, is rare in the Indian subcontinent. It presents as a brown, blue, or gray patch on the face and is within the distribution of the ophthalmic and maxillary branches of the trigeminal nerve. The oral cavity is infrequently involved in nevus of Ota. Only 11 cases have been documented in the English literature. We report a rare case of intraoral nevus of Ota in a 22-year-old male patient. This paper focuses on the differential diagnosis of oral manifestations of nevus of Ota to assist in proper followup to avert malignant transformation.

A rare metastasis from a rare brain tumour

DEFF Research Database (Denmark)

Aabenhus, Kristine; Hahn, Christoffer Holst

2014-01-01

This case report presents the story of a patient with an oligodendroglioma metastasizing to the bone marrow and to lymph nodes of the neck. The patient had undergone primary brain surgery 13 years prior to the discovery of metastases and radiotherapy directed at the brain tumour two months prior........ Oligodendroglioma are rare primary brain tumours of which extraneural metastasis is even more rare. The incidence of cases like this may be increasing because of better treatment and thus longer survival of patients with oligodendroglioma....

Rare case of nephrotic syndrome: Schimke syndrome.

Science.gov (United States)

Pedrosa, Anna Kelly Krislane de Vasconcelos; Torres, Luiz Fernando Oliveira; Silva, Ana Corina Brainer Amorim da; Dantas, Adrianna Barros Leal; Zuntini, Káthia Liliane da Cunha Ribeiro; Aguiar, Lia Cordeiro Bastos

2016-01-01

Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case. Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.

Cognitive-Behavioral Treatment of Hoarding in Youth: A Case Illustration.

Science.gov (United States)

McKay, Dean

2016-11-01

Hoarding in children is associated with more severe ancillary psychopathology, and has poor treatment outcome. At present, there are no empirically established procedures for treating hoarding in youth. The present case illustration is of a 10-year old child ("Grace") who presented for treatment with significant hoarding related to academic concerns and additional unrelated symptoms of obsessive-compulsive disorder (OCD). Grace was treated with cognitive behavior therapy (CBT) primarily comprising exposure with response prevention, behavioral experiments, and cognitive therapy, along with a program of reinforcement delivered by her parents to maintain her motivation for therapy. After 23 sessions and one booster session, Grace's symptoms improved significantly, with gains maintained at 1-year follow-up. In addition to the benefits of the specific interventions chosen, the role of therapist-patient/parent alliance as a contributory factor for good outcome is emphasized. As hoarding is underinvestigated in youth, suggestions for further investigation are offered. © 2016 Wiley Periodicals, Inc.

A rare case of haemolytic disease of newborn with Bombay phenotype mother

Directory of Open Access Journals (Sweden)

Shamee Shastry

2013-01-01

Full Text Available We are reporting a rare case of severe hemolytic disease of newborn (HDN with Bombay phenotype mother. A retrospective study of a case with severe haemolytic disease of newborn with Bombay phenotype mother was done. Blood grouping, antibody screening, and lectin study was done on the blood sample of the baby and mother to confirm the diagnosis. Hematological and biochemical parameters were obtained from the hospital laboratory information system for the analysis. Blood group of the baby was A positive, direct antiglobulin test was negative. Blood group of the mother was confirmed to be Bombay phenotype, Hematological parameters showed all the signs of ongoing hemolysis and the bilirubin level was in the zone of exchange transfusion. Due to the unavailability of this rare phenotype blood unit, baby was managed conservatively. Anticipating the fetal anemia and HDN with mothers having Bombay phenotype and prior notification to the transfusion services will be of great help in optimizing the neonatal care and outcome.

Radiology illustrated. Hepatobiliary and pancreatic radiology

International Nuclear Information System (INIS)

Choi, Byung Ihn

2014-01-01

Clear, practical guide to the diagnostic imaging of diseases of the liver, biliary tree, gallbladder, pancreas, and spleen. A wealth of carefully selected and categorized illustrations. Highlighted key points to facilitate rapid review. Aid to differential diagnosis. Radiology Illustrated: Hepatobiliary and Pancreatic Radiology is the first of two volumes that will serve as a clear, practical guide to the diagnostic imaging of abdominal diseases. This volume, devoted to diseases of the liver, biliary tree, gallbladder, pancreas, and spleen, covers congenital disorders, vascular diseases, benign and malignant tumors, and infectious conditions. Liver transplantation, evaluation of the therapeutic response of hepatocellular carcinoma, trauma, and post-treatment complications are also addressed. The book presents approximately 560 cases with more than 2100 carefully selected and categorized illustrations, along with key text messages and tables, that will allow the reader easily to recall the relevant images as an aid to differential diagnosis. At the end of each text message, key points are summarized to facilitate rapid review and learning. In addition, brief descriptions of each clinical problem are provided, followed by both common and uncommon case studies that illustrate the role of different imaging modalities, such as ultrasound, radiography, CT, and MRI.

Radiology illustrated. Hepatobiliary and pancreatic radiology

Energy Technology Data Exchange (ETDEWEB)

Choi, Byung Ihn (ed.) [Seoul National Univ. Hospital (Korea, Republic of). Dept. of Radiology

2014-04-01

Clear, practical guide to the diagnostic imaging of diseases of the liver, biliary tree, gallbladder, pancreas, and spleen. A wealth of carefully selected and categorized illustrations. Highlighted key points to facilitate rapid review. Aid to differential diagnosis. Radiology Illustrated: Hepatobiliary and Pancreatic Radiology is the first of two volumes that will serve as a clear, practical guide to the diagnostic imaging of abdominal diseases. This volume, devoted to diseases of the liver, biliary tree, gallbladder, pancreas, and spleen, covers congenital disorders, vascular diseases, benign and malignant tumors, and infectious conditions. Liver transplantation, evaluation of the therapeutic response of hepatocellular carcinoma, trauma, and post-treatment complications are also addressed. The book presents approximately 560 cases with more than 2100 carefully selected and categorized illustrations, along with key text messages and tables, that will allow the reader easily to recall the relevant images as an aid to differential diagnosis. At the end of each text message, key points are summarized to facilitate rapid review and learning. In addition, brief descriptions of each clinical problem are provided, followed by both common and uncommon case studies that illustrate the role of different imaging modalities, such as ultrasound, radiography, CT, and MRI.

Rogue-Elephant-Inflicted Panfacial Injuries: A Rare Case Report

Directory of Open Access Journals (Sweden)

Santosh Kumar Yadav

2012-01-01

Full Text Available Attacks by elephants, the largest of the “large animals,” produce many fatalities a year. Most attacks are provoked, although rogue elephants are occasionally responsible. Trampling, goring, tossing the individual with the trunk, or crushing with the knees produces the injuries. Injuries from encounters with large animals represent a significant health risk for rural communities. Wild-animal-inflicted maxillofacial injuries are rare, and limited literature is available describing their management. We present a case of severe maxillofacial injuries caused by the attack of a rogue elephant.

The use of quantum chemistry in pharmaceutical research as illustrated by case studies of indometacin and carbamazepine

DEFF Research Database (Denmark)

Gordon, Keith C; McGoverin, Cushla M; Strachan, Clare J

2007-01-01

A number of case studies that illustrate how quantum chemistry may be used in studying pharmaceutical systems are reviewed. A brief introduction to quantum methods is provided and the use of these methods in understanding the structure and properties of indometacin and carbamazepine is discussed...

A Rare Case of Rib Osteoblastoma: Imaging Features and Review of Literature

International Nuclear Information System (INIS)

Taghipour Zahir, Shokouh; Sefidrokh Sharahjin, Naser; Kargar, Saeed

2013-01-01

Osteoblastoma is a rare benign, but locally aggressive bone tumor with rare malignant transformation. It mostly affects the vertebral column and long bones. Radiographically, it is seen as an expansile, oval, sclerotic or lytic mass-like lesion with well-defined borders, although sometimes it may mimic a malignant tumor such as osteogenic sarcoma by its irregular borders. Herein, we report a case of osteoblastoma in a 22 year-old man with a long history of back and neck pain accompanied with neck stiffness. On the routine chest X-ray, the salient lesion appeared as an expansile, oval, sclerotic mass with well-defined borders and speckled calcification without any internal lucency and periosteal reaction, involving the posterolateral aspect of the first left thoracic rib, a rare anatomical site. Despite the unusual location, osteoblastoma should be considered in the differential diagnosis of a solitary rib lesion

Radiographic diagnosis of a rare case of oculo-dento-digital dysplasia

Directory of Open Access Journals (Sweden)

Umesh Chandra Parashari

2011-12-01

Full Text Available Oculodentodigital dysplasia (ODDD, also known as oculodento-osseous dysplasia, is an extremely rare autosomal dominant disorder with high penetrance, intra- and interfamilial phenotypic variability, and advanced paternal age in sporadic cases. The incidence of this disease is not precisely known, with only 243 cases reported in the scientific literature, suggesting an incidence of around 1 in 10 million people. It is marked mainly by eye abnormalities, craniofacial dysmorphism, dental anomalies, hand and foot malformations, various skeletal defects, and mildly delayed mental development. Neurological changes may appear earlier in each subsequent generation. This case report describes a radiological diagnosis of ODDD based on physical appearance, clinical features and radiographic findings in a 16-year-old girl.

Rare earth - no case for government intervention

OpenAIRE

Georg Zachmann

2010-01-01

China has officially restricted exports of rare earth for several years and announced this year it will further tighten exports. Rare earth is a group of 17 different metals, usually found clustered together. These metals have hundreds of different industry applications. For example, they are used in certain high capacity magnets, batteries and lasers. As the rare earth elements are used in sectors that are assumed to have an over-proportionate growth potential (eg. green-technology), policy ...

A Rare Case Report of Subcutaneous Mycoses by Rhytidhysteron Rufulum

Directory of Open Access Journals (Sweden)

Yadav Sarita

2017-04-01

Full Text Available Phaeohyphomycosis is a term to describe cutaneous and systemic or disseminated mycoses caused by a variety of dematiaceous fungi. Rhytidhysteron rufulum is a poorly known, common, pantropical phaeoid fungal species, and are rare human pathogens. Here, we describe a clinical case report of subcutaneous phaeohyphomycosis; to the best of our knowledge, this is the fourth report of infection with the Rhytidhysteron rufulum. A 54-year-old male presented to medicine outpatient department with complaints of respiratory distress. The patient was diagnosed as a case of chronic bronchitis with acute exacerbation. On examination, a well-defined soft subcutaneous swelling was detected on anterior aspect of right leg. The molecular identification revealed its homology with Rhytidhysteron rufulum. The phaeohyphomycotic infections are increasingly reported globally. This case highlights the need of awareness and high degree of suspicion for fungal aetiology, so that an early and adequate treatment can be given in such cases.

Pancreatitis and myocarditis followed by pulmonary hemorrhage, a rare presentation of leptospirosis- A case report and literature survey

Directory of Open Access Journals (Sweden)

Ranawaka Nuwan

2013-01-01

Full Text Available Abstract Background Leptospirosis is a potentially fatal disease which can cause multi-organ dysfunction. It can rarely present as acute pancreatitis. This is the first ever report of leptospirosis presenting with acute pancreatitis and myocarditis followed by diffuse pulmonary hemorrhages to the best of our knowledge. Case presentation A 15-year-old South Asian boy presented with high grade fever, epigastric discomfort and was anicteric on admission. He developed tachycardia, transient hypotension, changes of electro-cardiogram and positive troponin I suggestive of myocarditis. Acute pancreatitis was diagnosed with 12 fold high serum amylase and with the evidence of computerized tomography. Then he developed diffuse pulmonary hemorrhages and later acute renal failure. Leptospirosis was confirmed by positive leptospira IgM, negative IgG and strongly positive Microscopic Agglutination Test. Other possible infective and autoimmune causes were excluded. Patient recovered completely with antibiotics and the supportive care. Conclusion This case illustrates diagnostic difficulties especially in resource poor settings where leptospirosis is common. Additionally it highlights the fact that leptospirosis should be considered in patients presenting with pancreatitis which can be complicated with myocarditis and diffuse pulmonary hemorrhages. We hypothesize that Toll like receptors may play a role in such systemic involvement.

Rare association of anophthalmia, complex congenital heart disease and pulmonary hypertension: case report.

Science.gov (United States)

Ríos-Méndez, Raúl Enrique; Lozano Chinga, Michell Marola

2016-10-07

Clinical congenital anophthalmia is described as the uni- or bilateral absence of the eyeball that might occur in isolation or as part of a syndrome. It has a very low prevalence and its etiology is heterogeneous. Complex congenital cardiac malformations are also rare. The association of congenital anophthalmia and congenital heart disease is rarer still, and the etiology of those associations is not well understood yet. We report the case of a patient who had the very rare association of bilateral anophthalmia, multiple cardiac malformations and severe pulmonary hypertension.

Case studies illustrating in-situ remediation methods for soil and groundwater contaminated with petrochemicals

Energy Technology Data Exchange (ETDEWEB)

Dixon, Robert A; Lance, P E; Downs, A; Kier, Brian P [EMCON Northwest Inc., Portland, OR (United States)

1994-12-31

Four case studies of successful in-situ remediation are summarized illustrating cost-effective methods to remediate soil and groundwater contaminated with volatile and non-volatile petrochemicals. Each site is in a different geologic environment with varying soil types and with and without groundwater impact. The methods described include vadose zone vapor extraction, high-vacuum vapor extraction combined with groundwater tab.le depression, air sparging with groundwater recovery and vapor extraction, and bio remediation of saturated zone soils using inorganic nutrient and oxygen addition

Case studies illustrating in-situ remediation methods for soil and groundwater contaminated with petrochemicals

Energy Technology Data Exchange (ETDEWEB)

Dixon, Robert A.; Lance, P.E.; Downs, A.; Kier, Brian P. [EMCON Northwest Inc., Portland, OR (United States)

1993-12-31

Four case studies of successful in-situ remediation are summarized illustrating cost-effective methods to remediate soil and groundwater contaminated with volatile and non-volatile petrochemicals. Each site is in a different geologic environment with varying soil types and with and without groundwater impact. The methods described include vadose zone vapor extraction, high-vacuum vapor extraction combined with groundwater tab.le depression, air sparging with groundwater recovery and vapor extraction, and bio remediation of saturated zone soils using inorganic nutrient and oxygen addition

Loss-of-benefits analysis for nuclear power plant shutdowns: methodology and illustrative case study

International Nuclear Information System (INIS)

Peerenboom, J.P.; Buehring, W.A.; Guziel, K.A.

1983-11-01

A framework for loss-of-benefits analysis and a taxomony for identifying and categorizing the effects of nuclear power plant shutdowns or accidents are presented. The framework consists of three fundamental steps: (1) characterizing the shutdown; (2) identifying benefits lost as a result of the shutdown; and (3) quantifying effects. A decision analysis approach to regulatory decision making is presented that explicitly considers the loss of benefits. A case study of a hypothetical reactor shutdown illustrates one key loss of benefits: net replacement energy costs (i.e., change in production costs). Sensitivity studies investigate the responsiveness of case study results to changes in nuclear capacity factor, load growth, fuel price escalation, and discount rate. The effects of multiple reactor shutdowns on production costs are also described

Primary Leiomyosarcoma of the Breast: A Rare Case Report

Directory of Open Access Journals (Sweden)

Pallavi Agrawal

2015-08-01

Full Text Available Background: Primary leiomyosarcoma (LMS of the breast is a very rare neoplasm of the breast arising from the mesenchymal tissue. The clinical presentation of this entity simulates other benign and malignant lesions of mesenchymal tissue of the breast.Case presentation: Case 1: A 40-year-old female presented with a right-sided breast lump, which was suspected to be a malignant spindle cell tumor on needle core biopsy (NCB. A multi-disciplinary team performed modified radical mastectomy (MRM with axillary node dissection on the patient with no post-operative chemo-radiation. Case 2: A 70-year-old female presented with a left sided breast lump and a palpable axillary node. Needle core biopsy diagnosed it as malignant spindle cell tumor. The patient underwent MRM with axillary node dissection. It was confirmed to be a case of breast LMS with axillary nodal metastasis. Both patients were followed up for one year with no evidence of recurrence.Conclusion: Both cases underwent MRM with axillary node dissection in our study. However, the role of axillary dissection in the prognosis and disease-free survival of the patients with primary LMS of the breast with axillary metastasis has not been studied yet. The optimal management of this entity remains to be tumor excision with clear margins.

A rare case of post-splenectomy gastric volvulus managed by laparoscopic anterior gastropexy

Directory of Open Access Journals (Sweden)

Rahul Amreesh Gupta

2017-01-01

Full Text Available We report an extremely rare case of recurrent gastric volvulus after open splenectomy for hereditary spherocytosis. The initial episode was managed by endoscopic derotation. Later, for recurrent symptoms, she was successfully managed by laparoscopic anterior gastropexy.

Dual malignancy in adolescence: A rare case report of metachronous papillary carcinoma of thyroid following dysgerminoma of ovary

Science.gov (United States)

Chakrabarti, Suvadip; Desai, Sanjay M.; Mehta, Dharmendra Y.; Somanath, Shreyas

2016-01-01

Dual malignancy is rare in adolescents. Dual malignancy with the second malignancy of thyroid is rare. No association has been reported between dysgerminoma of ovary and carcinoma thyroid in medical literature. Despite a thorough PubMed search (key words — Papillary carcinoma of thyroid, metachronous, dysgerminoma ovary), we were unable to find a previous reported case of metachronous papillary carcinoma of thyroid (PTC) following dysgerminoma of the ovary. After surgery, the patient is being regularly followed up for recurrence/development of new primary. We report this unusual and rare case in a 17-year-old female patient. PMID:27904567

Dual malignancy in adolescence: A rare case report of metachronous papillary carcinoma of thyroid following dysgerminoma of ovary

Directory of Open Access Journals (Sweden)

Suvadip Chakrabarti

2016-01-01

Full Text Available Dual malignancy is rare in adolescents. Dual malignancy with the second malignancy of thyroid is rare. No association has been reported between dysgerminoma of ovary and carcinoma thyroid in medical literature. Despite a thorough PubMed search (key words - Papillary carcinoma of thyroid, metachronous, dysgerminoma ovary, we were unable to find a previous reported case of metachronous papillary carcinoma of thyroid (PTC following dysgerminoma of the ovary. After surgery, the patient is being regularly followed up for recurrence/development of new primary. We report this unusual and rare case in a 17-year-old female patient.

A rare case of hypocalcemia induced by nilotinib

Directory of Open Access Journals (Sweden)

Marija Petrić

2017-03-01

Full Text Available Chronic myelogenous leukemia (CML is a myeloproliferative disorder characterized by increased proliferation of predominantly myeloid cells in the bone marrow and their accumulation in the peripheral blood. Nowadays, drugs known as tyrosine kinase inhibitors (TKIs are the standard treatment for CML. Since TKIs specifically target BCR-ABL, the activated tyrosine kinase fusion protein, they are expected to cause less hematological and nonhematological side effects than medications used before. We report a case of a 61-year-old patient treated with the second-generation TKI, nilotinib, that presented with very common side effects (skin rash, myalgia, and paresthesia, and also with rare, but severe hypocalcemia and potentially dangerous and fatal QTc elongation.

Primary Bladder Neurofibroma: A Rare Case with Clinical Implications and Diagnostic Challenges.

Science.gov (United States)

Umakanthan, Srikanth; Naik, Ramadas; Bukelo, Maryann Margaret; Rai, Sharada; Prabhu, Laxman

2015-09-01

Neurofibroma of the genito-urinary tract is rare. Urinary bladder is the commonest organ involved in cases of urinary tract involvement. Patients present early in life and there is male preponderance. We discuss here a case of primary neurofibroma of the urinary bladder in a 52-year-male presenting with haematuria, irritative bladder symptoms and pelvic mass. Cystoscopy showed a swelling in the left lateral wall. A transurethral biopsy revealed neurofibroma of the urinary bladder. Immunohistochemical studies confirmed the diagnosis.

SMART Syndrome: Case report of a rare complication after cerebral radiation therapy

International Nuclear Information System (INIS)

Truntzer, P.; Salze, P.; Monjour, A.; Gaultier, C.; Ahle, G.; Guillerme, F.; Boutenbat, G.; Atlani, D.; Stilhart, B

2012-01-01

The authors report a 71-year-old woman case who developed, 7 years after a cerebral radiation therapy for a parieto-occipital glioblastoma, a stroke-like migraine attacks after radiotherapy syndrome (SMART syndrome), a rare complication characterized by reversible neurologic deficits with migraine described after cerebral irradiation. Transient gyri-form reversible enhancement is found on MRI during crises. This case report allows discussing the clinical, iconographic presentation and the clinical outcome of this syndrome at the light of the literature publication. (authors)

Evaluation of School Psychological Services: A Case Illustration.

Science.gov (United States)

Fairchild, Thomas N.; Seeley, Tracey J.

1996-01-01

Illustrates how school psychological services were evaluated in two elementary schools. Questionnaires were used to solicit input from students regarding individual counseling and classroom guidance activities. Rating scales were used to solicit input from parents regarding the assessment service and from building staff regarding their perception…

An Unsuspected Finding of t(9;22: A Rare Case of Philadelphia Chromosome-Positive B-Lymphoblastic Lymphoma

Directory of Open Access Journals (Sweden)

Prajwal Boddu

2017-01-01

Full Text Available While rare, cases of isolated extramedullary disease of B-cell Lymphoblastic Lymphoma (B-LBL without morphologic bone marrow involvement have been described. In this report, we illustrate the case of an elderly gentleman who presented with isolated testicular and vertebral LBL involvement but had no morphologic bone marrow involvement. The initial plan of treatment was to treat along the lines of Philadelphia negative B-ALL/LBL. During this time, fluorescence in situ hybridization (FISH and PCR testing for BCR-ABL1 rearrangements were being performed on the marrow specimens as a part of routine diagnostic workup. While the FISH returned negative, PCR testing unexpectedly detected BCR-ABL1 fusion transcripts at a low level of 0.48%. Given their presence, we performed FISH for BCR/ABL1 rearrangement in both testicular and L5 vertebral specimens which were 80–90% positive. He subsequently received rituximab, hyper-CVAD, and dasatinib, along with prophylactic intrathecal prophylactic chemotherapy. The patient achieved a prolonged remission but eventually relapsed, 4 years later. Had it not been for this fortuitous discovery, the patient would not have been treated with tyrosine kinase inhibitors. We emphasize that FISH and PCR testing for BCR-ABL1 rearrangement are integral to arriving at an accurate diagnosis and should be routinely tested on B-LBL biopsy specimens.

An aggressive angiomyxoma of vulva - A rare entity - A case report

Directory of Open Access Journals (Sweden)

Bishnu Prasad Das

2016-01-01

Full Text Available Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor predominantly presenting in women of reproductive age and also having a moderate-to-high risk for local relapse. Hence, it needs to be differentiated from other mesenchymal tumors occurring in this region. We present here a case of a 40-year-old female presenting with a large, fleshy, pedunculated mass on the right labia majora.

Ovarian tubercular abscess mimicking ovarian carcinoma: A rare case report

Directory of Open Access Journals (Sweden)

Abinash Agarwala

2015-01-01

Full Text Available Although genito-urinary tuberculosis is common, reports of isolated ovarian tubercular abscess are rare. Ovarian tubercular abscess may mimics that of an ovarian tumor, leading to diagnostic difficulties. We reported a case report of 35 years woman presented with chronic pain abdomen, weight loss, low-grade fever and a right ovarian mass on ultrasound, with a significantly elevated CA-125 level. On clinical and radiological evidence, diagnosis of ovarian carcinoma was made, and laparotomy was performed with resection of the ovary. Postoperative specimen sent for histological examination that revealed classic epithelioid granuloma and acid-fast bacilli were present in Ziehl-Neelsen stain. Patient was put on antitubercular regimen from our Dots center. She is improving clinical after taking antitubercular drug and is on regular follow up at our chest outpatient department. Ovarian tubercular abscess is common in young women living in endemic zones, but case report of isolated tubercular abscess is rarely reported. CA-125 can be raised in both ovarian tubercular abscess and ovarian carcinoma, and only imaging is not always conclusive. Laparotomy followed by tissue diagnosis can be helpful in this situation. As the prognosis and treatment outcome of ovarian tubercular abscess and ovarian carcinoma is different, proper diagnosis by laparotomy should be done. Early diagnosis of ovarian tubercular abscess is vital as untreated disease can lead to infertility.

A RARE CASE OF BILATERAL MICROSPHEREPHAKIA

Directory of Open Access Journals (Sweden)

Pandu

2015-05-01

Full Text Available Microspherophakia is rare bilateral congenital anamoly of the crystalline lens. The condition may be isolated , familial or it may be associated with systemic affections like Marfan's syndrome , Weil - Marchesani syndrome , hyperlysinemia and congenital rubella. Microspherophakia results in lenticular myopia , lens dislocation , usually inferiorly and inverse glaucoma. We present a case in a 8 year old child who presented with bilateral microspherophakia and anterior dislocation of lens of right eye. visual acuity in right eye was counting fingers close to face and i n left eye 6/60.IOP with perkins applanation tonometer was 30mmHg in right eye 22mmHg in left eye , cornea was hazy due to edema , anterior chamber was shallow in both eye patient was managed with emergency lens extraction of right eye and secondary ACIOL im plantation. Left eye was managed by laser peripheral iridotomy. IOP was within normal limits postoperatively in both eyes without any antiglaucoma medications. Postoperatively best corrected visual acuity in right was 6/18 and 6/9 in left eye.

Retroperitoneal Malignant Peripheral Nerve Sheath Tumour: A Rare Case Report.

Science.gov (United States)

Deger, Ayse Nur; Bayar, Mehmet Akif; Caydere, Muzaffer; Deger, Hakki; Tayfur, Mahir

2015-09-01

Malignant nerve sheath tumours (MPNST) are rare neoplasias and retroperitoneal cases are fairly rare and clinically difficult to be detected, but they are very agressive neoplasias. MPNST are frequently seen in head, neck and upper extremities. In patients with NF1; MPNST, a poor-prognostic lesion, may result from a malignant degeneration of a former plexiform neurofibroma. It is necessary to be aware of a potential malignancy in patients diagnosed with plexiform neurofibroma. We present a 21-year-old female with a diagnosis of MPNST. The patient was admited to the hospital because of a tumour in the subcutaneous region on her left buttock. The surgeon's clinical diagnosis was lipoma. After the pathological examination of biopsy specimen, the lesion was identified as "plexiform neurofibroma" and then the patient was diagnosed with Neurofibromatosis Type 1 (NF1). Simultaneously, another mass on the retroperitoneal region was identified as malignant peripheral nerve sheath tumour (MPNST).

A case report of gastric lymphocytic phlebitis, a rare mimic for malignancy.

Science.gov (United States)

Chan, Daniel L; Ravindran, Praveen; Chua, Dorothy; Smith, Jason D; Wong, King S; Ghusn, Michael A

2017-01-01

Lymphocytic phlebitis is a benign condition characterised by inflammation of the veins and rarely affects the gastrointestinal tract. Reported cases present as acute abdomen and involve the colon or small intestine. We report the fourth case of gastric lymphocytic phlebitis in the literature. A 74-year-old female presented with eight weeks of abdominal pain. Findings at endoscopy were suggestive of a malignant ulcer on the greater curvature of antrum, while biopsies showed chronic gastritis without malignancy. Appearance at diagnostic laparoscopy was consistent with a malignant gastric ulcer with serosal changes. Due to persistent pain and the macroscopic appearance, she proceeded to have an open subtotal gastrectomy and D2 lymph node clearance. Despite macroscopic appearance, the microscopic examination demonstrated no malignancy, and was consistent with lymphocytic phlebitis with overlying ulceration. This case was a mimic for gastric malignancy, with the benign diagnosis only being made after surgical resection. Gastric lymphocytic phlebitis is a rare differential diagnosis for gastric ulcers when biopsies are negative, although preoperative diagnosis is difficult given the lesions do not involve the mucosa. If clinical history and endoscopic findings are suspicious for malignancy, despite normal biopsies, an aggressive surgical resection remains reasonable given the rarity gastric lymphocytic phlebitis. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

Xeroderma pigmentosum: A rare case report with review of literature

Directory of Open Access Journals (Sweden)

B Anand

2012-01-01

Full Text Available Xeroderma pigmentosum, or XP, is an autosomal recessive genetic disorder in which the ability to repair DNA damage caused by ultraviolet (UV light is deficient. In extreme cases, all exposure to sunlight must be forbidden, no matter how small. As such, individuals with the disease are often colloquially referred to as ′Children of the Night′. Mutations in XP genes that regulate nucleotide excision repair, not only predispose persons with xeroderma pigmentosum to multiple malignancies, but also promote premature cutaneous and ocular ageing, and in some cases promote progressive neurodegenerative changes. There is a great involvement of many parts of the body, especially head and neck. The oral manifestations are mainly related to the occurrence of malignant tumors in the lips, tongue and buccal mucosa. This paper reports a rare case of XP in a 40-year-old female presenting with dermatological, oral and ophthalmological involvement.

Malignant Hidradenocarcinoma in the Lower Extremity: A Case Report of a Rare Tumor.

Science.gov (United States)

Kane, Brendan; Adler, Evan; Bhandari, Tarun; Rose, Michael; DiGuglielmo, Nicola; Sun, Xiu

Malignant hidradenocarcinomas are rare soft tissue tumors of sweat gland origin. We present the case of a soft tissue, fungating tumor of 15 years' duration of the medial ankle in an 85-year-old male that exhibited malignant features clinically and radiographically. Subsequent punch biopsy revealed a diagnosis of malignant hidradenocarcinoma. Given the risk of recurrence and the poor radiation and chemotherapy options, the patient initially decided to leave the lesion untreated. However, he soon developed lower extremity cellulitis from the exposed lesion and decided to have the tumor excised, eliminating the source of the infection. In the present case study, we discuss the etiology, clinical and radiographic characteristics, and treatment options for this rare lesion. At the 18-month follow-up visit, he had had no recurrence of the lesion. Copyright © 2017 The American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

Lumbosacral arachnoid cyst with tethered cord: A rare case report

Directory of Open Access Journals (Sweden)

S K Jain

2012-01-01

Full Text Available Arachnoid cysts are cerebrospinal fluid collections in the spine that can present with neurological symptoms or be discovered accidentally. Intradural location of such cysts especially in the lumbosacral region is relatively rare. The association of such cysts with other congenital anomalies such as tethered cord lends evidence to the developmental origin of arachnoid cysts. We report a case of lumbosacral arachnoid cyst with tethered cord in a 6-year-old male child and discuss the etiopathogenesis and management options.

Hydatid disease of the spine: A rare case

Directory of Open Access Journals (Sweden)

Mona Agnihotri

2017-01-01

Full Text Available Hydatid disease or hydatidosis is the most widespread zoonosis caused by Echinococcus granulosus. Liver and lungs are the most common sites. Bone involvement is rare and reported in 0.5%–4% with spinal involvement reported in 50% of these cases. We present a case of spinal hydatidosis in a 35-year-old male presenting with lower extremity weakness and numbness. Magnetic resonance imaging (MRI of the spine showed multiple cystic lesions at the T9–T11 level with involvement of the paraspinal muscles. The lesion was seen intraspinal, intradural, intramedullary, and epidural. Radiological impression was aneurysmal bone cyst. The patient underwent laminectomy, and the excised cysts showed characteristic features of hydatid cyst (HC on histopathology. The patient was started on antihelminthic therapy postoperatively. MRI is a diagnostic modality for HC, but the unusual location and absence of characteristic features can cause diagnostic difficulty. A high index of suspicion should be kept in patients residing in endemic areas and presenting with unusual cystic lesion of spine.

A rare case of metastatic squamous urachal carcinoma.

Science.gov (United States)

Andrei, S; Andrei, A; Rusu Muntean, G; Ungureanu, M; Herlea, V; Becheanu, G; Popescu, I

2013-01-01

Squamous cell carcinoma is a very rare type of urachal malignancy, only a few cases being reported in the medical literature. We present the case of a 49-year-old male patient diagnosed with infected squamous cell urachal carcinoma with multiple pulmonary metastases, after complaints of lower abdominal pain, abdominal mass and fever, without respiratory symptoms. The abdominal ultrasonography and the CT scan revealed a tumoral mass in the lower abdomen in contact with the abdominal wall and the urinary bladder dome, displacing the small bowel. Pulmonary nodular lesions were described in the left lobe pyramid. The intraoperative diagnosis was necrotic urachal tumor with urinary bladder dome invasion and suspected pulmonary metastases, and tumor ablation with bladder dome resection and suture of the bladder were performed. The histopathological result was poorly differentiated squamous cell carcinoma (G3), with negative resection margins. The patient recovered well after surgery, but the prognosis is very poor due to the metastatic stage in which the tumor was diagnosed, no standard chemotherapy regimen for the treatment of metastatic urachal carcinoma being known as effective until now. Celsius.

A Rare Case of Gestational Gigantomastia with Hypercalcemia: The Challenges of Management and Follow up

Science.gov (United States)

Moazzami, Bahram; Chaichian, Shahla; Farahvash, Mohammad Reza; Taheri, Saeedeh; Ahmadi, Seyed Ali; Mokhtari, Majid; Sheibani, Kourosh

2016-01-01

Background: Gigantomastia is a breast disorder marked by exaggerated rapid growth of the breasts, generally bilaterally. Since this disorder is very rare and has been reported only in sparse case reports its etiology has yet to be fully established. Treatment is aimed at improving the clinical and psychological symptoms and reducing the treatment side effects; however, the best therapeutic option varies from case to case. Case Presentation: The present report described a case of gestational gigantomastia in a 30-year-old woman, gravida 2, parity 1, 17 week pregnant admitted to Pars Hospital, Tehran, Iran, on May 2014. The patient was admitted to hospital at week 17 of pregnancy, although her breasts initially had begun to enlarge from the first trimester. The patient developed hypercalcemia in her 32nd week of pregnancy. The present report followed this patient from diagnosis until the completion of treatment. Conclusion: Although gestational gigantomastia is a rare condition, its timely prognosis and careful examination of some conditions like hyperprolactinemia and hypercalcemia is essential in successful management of this condition. PMID:27921004

Giant cemento-ossifying fibroma of the mandible: a rare case.

Science.gov (United States)

Sujatha, D; Shubha, Gurudath; Anuradha, Pai; Ragavendra, Mahadev Naik

2013-01-01

The World Health Organization classifies cemento-ossifying fibroma (COF) as a fibro-osseous neoplasm included among the nonodontogenic tumors derived from the mesenchymal blast cells of the periodontal ligament, with a potential for forming fibrous tissue, cementum and bone, or a combination of such elements. These are slow-growing lesions, and are more frequent in women, between the third and fourth decades of life. Case reports of massive expansile COFs (measuring more than 10 cm) are rarely reported in the literature. This article aims to describe a case of giant cemento-ossifying fibroma with radiographic and 3D CT features in a 34 year old female patient, who came with the complaint of progressive swelling of the face which had started 6 years earlier.

Ectopic third molar in maxillary sinus: A rare case report

Directory of Open Access Journals (Sweden)

Abhishek Sinha

2017-01-01

Full Text Available Ectopic tooth eruption in a non-dental area is a rare entity, and is most common in oral cavity. There have been a few case reports of teeth erupting in mandibular condyle, chin, palate, coronoid process, and maxillary sinus. Ectopic tooth in the maxillary sinus are found incidentally on routine radiological examination, same time they can be symptomatic and associated with pathologies usually dentigerous cyst or odontogenic keratocyst. Facial pain, purulent rhinorrhoea, epistaxis, headache, swelling, and epiphora-related naso-lacrimal duct obstruction can also be seen. By Caldwell-Luc procedure the ectopic teeth within the maxillary sinus are often removed. In this study, a case of ectopic maxillary third molar tooth on right maxillary sinus is presented.

Spontaneous successful pregnancy in posthypophysectomy hypopituitarism: A rare case report

Directory of Open Access Journals (Sweden)

Indu Lata

2014-01-01

Full Text Available Pregnancy in patients with pan-hypopituitarism following surgery of pituitary adenoma is rare and considered high risk. Hormonal dysfunction in these patients involves more than one axis (gonadotrophic, thyroidal, and adrenal. However, advance in infertility treatment have led to the increased pregnancy rate in hypopituitarism women. We present a case of nonfunctioning pituitary macroadenoma, who after pituitary surgery (hypophysectomy developed hypopituitarism followed by multiple tuberculoma brain with hydrocephalus with arachnoiditis. She conceived spontaneously after 9 years of pituitary surgery and carried her pregnancy to the term. Elective caesarean section was done at 38 weeks and both infant and mother are well. The case highlights the rarity of the phenomenon and the safe outcome of the pregnancy with proper replacement.

Guillain Barre Syndrome Following Traumatic Brain Injury: A Rare Case

Directory of Open Access Journals (Sweden)

Kirac Unal

2016-06-01

Full Text Available Introduction Guillain-Barre syndrome (GBS is an immune-mediated acute inflammatory disorder of the peripheral nervous system. Infectious agents were usually accused of playing a role in the etiology of GBS. Guillain-Barre syndrome has rarely been reported following subdural and subarachnoid hemorrhage after head trauma. Case Presentation We report on a 63-year-old male patient presenting GBS following Traumatic Brain Injury (TBI. Only five other similar cases are described in the literature. Conclusions Sudden onset of GBS symptoms following trauma may erroneously be assessed as secondary complications of the TBI and can lead to unnecessary procedures such as computerized tomography (CT scan and magnetic resonance imaging (MRI for a definitive diagnosis and may be a waste of time.

Primary tuberculosis of the breast manifested as abscess: a rare case report.

Science.gov (United States)

Gupta, Samita; Singh, Vikram J; Bhatia, Gaurav; Dhuria, Kshitiz

2014-01-01

Primary breast tuberculosis is a rare entity. We are reporting a case of primary breast tuberculosis, which presented as breast abscess. On histopathology, it was diagnosed as breast tuberculosis. Aspiration cytology was not done due breast abscess. Patient was put on anti-tubercular drugs. In follow up, after 3 months patient condition was improved.

Developmentally sensitive cognitive behavioral therapy for adolescent school refusal: rationale and case illustration.

Science.gov (United States)

Heyne, David; Sauter, Floor M; Ollendick, Thomas H; Van Widenfelt, Brigit M; Westenberg, P Michiel

2014-06-01

School refusal can be difficult to treat and the poorest treatment response is observed among older school refusers. This poor response may be explained, in part, by the impact of developmental transitions and tasks upon the young person, their family, and the treatment process. This paper describes and illustrates the @school program, a cognitive behavioral therapy (CBT) designed to promote developmental sensitivity when planning and delivering treatment for adolescent school refusal. Treatment is modularized and it incorporates progress reviews, fostering a planned yet flexible approach to CBT. The treatment is illustrated in the case of Allison, a 16-year-old female presenting with major depressive disorder and generalized anxiety disorder. A case formulation guided the selection, sequencing, and pacing of modules targeting predisposing, precipitating, perpetuating, and protective factors. Treatment comprised 16 sessions with Allison (interventions addressing depression, anxiety, and school attendance) and 15 concurrent sessions with her mother (strategies to facilitate an adolescent's school attendance), including two sessions with Allison and mother together (family communication and problem solving to reduce parent-adolescent conflict). Two treatment-related consultations were also conducted with Allison's homeroom teacher. Allison's school attendance improved during the course of treatment. By post-treatment, there was a decrease in internalizing behavior, an increase in self-efficacy, and remission of depressive disorder and anxiety disorder. Clinically significant treatment gains were maintained at 2-month follow-up. Factors influencing outcome may include those inherent to the @school program together with less specific factors. Special consideration is given to parents' use of both authoritative and autonomy-granting approaches when helping an adolescent to attend school.

Clozapine-induced interstitial nephritis - a rare but important complication: a case report

Directory of Open Access Journals (Sweden)

Hunter Robert

2009-08-01

Full Text Available Abstract Introduction Given the limited range of effective drug treatments for patients with schizophrenia, increasing numbers of patients, often termed 'treatment-resistant' are prescribed clozapine. While the induction of neutropenia or agranulocytosis by clozapine is well appreciated, other rare potentially fatal adverse reactions may also occur including acute interstitial nephritis as reported in this case. Case presentation A 57-year-old Caucasian woman with treatment-resistant chronic schizophrenia developed acute renal failure following initiation of treatment with clozapine. The adverse reaction occurred after only four doses of the drug had been administered (titrated from 12.5 to 25 mg per day. After clozapine had been withdrawn, the patient's renal function returned to normal with no other changes to medication. The patient had been exposed to clozapine about 4 years previously when she had developed a similar reaction. Conclusion Renal reactions to clozapine are extremely rare but, if not recognized promptly, may prove fatal. Psychiatrists need to be aware of this possible complication when clozapine is initiated.

Primary laryngeal leishmaniasis: A rare case report

Directory of Open Access Journals (Sweden)

Kumar Bipin

2009-01-01

Full Text Available Laryngeal leishmaniasis is extremely rare. We report a case of primary laryngeal leishmaniasis in a 70-year-old male who was admitted with complaints of gradual progressive hoarseness of the voice, dyspnea, cough for the past 3 months and noisy breathing for the past 5 days. An X-ray of the soft tissue of the neck showed a prevertebral soft tissue mass causing narrowing of the airway at the C6-C7 vertebral level. A computerized tomography (CT scan showed a soft tissue mass in the subglottic region causing significant narrowing of the airway. A direct laryngoscopy showed a pinkish-white, friable mass involving the subglottic region and the anterior half of the vocal cords. With the clinical suspicion of malignancy, an endoscopic biopsy was done. A histopathological examination showed diffuse mixed inflammatory cell infiltrate in subepithelium with numerous Leishmania donovani bodies in the cytoplasm of histiocytes.

A Rare Case of Recurrent Myoid Hamartoma Mimicking Malignancy: Imaging Appearances

International Nuclear Information System (INIS)

Ko, Myung Su; Jung, Won Sang; Choi, Hyun Joo; Cha, Eun Suk

2010-01-01

Myoid hamartoma is an uncommon type of breast hamartoma and its recurrence is very rare. We report the imaging appearance of an unusual case of recurrent myoid hamartoma of the breast mimicking malignancy in a 43-year-old woman. Although the mammographic and ultrasonographic findings have long been described in the literature, MR finding with a dynamic study has not, to the best of our knowledge, been reported previously

Plasma cell gingivitis - A rare case related to Colocasia (arbi) leaves.

Science.gov (United States)

Bali, Deepika; Gill, Sanjeet; Bali, Amit

2012-09-01

Plasma cell gingivitis is an uncommon inflammatory condition of uncertain etiology often flavoured chewing gum, spices, foods, candies, or dentifrices. The diagnosis of plasma cell gingivitis is based on comprehensive history taking, clinical examination, and appropriate diagnostic tests. Here we are presenting a rare case of plasma cell gingivitis caused by consumption of colocasia (arbi) leaves. Colocasia is a kind of vegetable, very commonly consumed in the regions of North India.

Mycotic aneurysm of the posterior tibial artery – a rare complication of bacterial endocarditis: a case report

Directory of Open Access Journals (Sweden)

Patel S

2008-11-01

Full Text Available Abstract Introduction Distal arterial embolisation and subsequent aneurysm formation are rare occurrences and most are secondary to trauma. We have found no case reports that describe posterior tibial aneurysm formation secondary to bacterial endocarditis. Case presentation We report the case of a 47-year-old Caucasian man who, 2 years after an episode of subacute bacterial endocarditis, presented with signs and symptoms consistent with posterior tibial aneurysm formation. Conclusion Posterior tibial aneurysm formation is a rare occurrence, most commonly occurring after trauma and, although other causes have been described, to our knowledge, endocarditis has not been implicated before, and as such should therefore be borne in mind when dealing with cases where no obvious aetiology is evident.

Social sustainability in supply chains: A framework and a Latin America illustrative case

Directory of Open Access Journals (Sweden)

Dafne Oliveira Carlos de Morais

2017-12-01

Full Text Available Social issues are under-represented in sustainability, considering historical predominance of economic and environmental issues. This also applies to Sustainable Supply Chain Management. Even with its definition clarified regarding Triple Bottom Line, research still advances disproportionately in environmental and economic dimensions, facing the social dimension. This research aims to analyze how social sustainability is addressed in focal firms and managed into its supply chain. The study explores the concepts of social issues and governance mechanisms, presenting elements discussed in the literature. A framework for managing social sustainability in supply chains is presented, followed by a case to illustrate the discussed concepts in a Latin American context.

Imaging of Kaposi sarcoma in a transplanted liver: A rare case report

Directory of Open Access Journals (Sweden)

Saumya Gupta

2015-06-01

Full Text Available In post-transplant patients, de novo malignancies such as post-transplant lymphoproliferative disease (PTLD, lung carcinoma, renal cell carcinoma, cutaneous malignancies, and Kaposi sarcoma are now seen. The immunotherapy used to prevent graft failure indirectly increases their risk. We present a rare case of visceral Kaposi sarcoma in a patient with orthotopic liver transplant.

Rare case of solitary plasmacytoma of the skull in a young male ...

African Journals Online (AJOL)

Solitary plasmacytoma of bone without signs of multiple myeloma is a rare entity. It usually presents as an osteolytic lesion in the axial skeleton of an elderly patient. Here, we report a case of solitary plasmacytoma in the skull of a young male patient which emphasises the need to consider it in the differential diagnosis of a ...

Ectopic Compound Odontoma in the Buccal Mucosa: Report of a Rare Case

Directory of Open Access Journals (Sweden)

Aparna Venigalla

2015-01-01

Full Text Available Eruption of tooth into extraosseous locations is an extremely rare condition. We report a case of a six-year-old girl child with tooth-like structure erupting from the right buccal mucosa. Clinical, radiographic, and histopathologic examination suggested the diagnosis of compound odontoma. Very few cases have been reported so far, where tooth has been located completely in the soft tissue and a variety of names have been used for that condition. A brief review of the literature and the ambiguity in naming the situation is discussed.

Bilateral breast metastases of a renal carcinoma: a case report and review of the literature.

Science.gov (United States)

Ganapathi, S; Evans, G; Hargest, R

2008-01-01

Metastatic tumours account for mastectomy and excision of right breast lump was done. Histology of both lesions confirmed them as metastatic deposits. Bilateral breast metastasis from a renal cancer is very rare and this is the second reported case. This case illustrates the potential for rare sites of metastases and for the consideration of metastasis in the presence of previous renal cancer. Recognition as metastatic neoplasm is important to prevent unnecessary radical procedures.

Non-Hodgkin's lymphoma of the mandible in HIV patient - A Rare Case Report

Directory of Open Access Journals (Sweden)

Mahesh Neerupakam

2018-01-01

Full Text Available Non-Hodgkin's lymphoma (NHL is a lymphatic system tumor originating from either B or T lymphocytes and shows a high malignant potential. In HIV-seropositive patients, NHL of head and neck is mainly found in Waldeyer's ring, oral mucosa, salivary glands, paranasal sinuses, and laryngeal tissue. Primary NHL rarely affects the bone. When the lesion affects the bones of the jaws, it is rare in the mandible when compared to the maxilla. In the reported cases, only 0.6% are found in the mandible. NHL of the mandible can be difficult to diagnose, and so the prime aim of the present case report is to establish appropriate diagnosis of one of such kinds. Clinically, they may imitate a dental infection with symptoms of pain and discomfort. A delay in diagnosis may lead to a poor prognosis. Herewith, we present a case of NHL on the lower-right mandible in a 40-year-old male. A correlation of clinical findings, radiological examination, and histopathological examination enabled us in early diagnosis and differentiating it from other similar conditions, thus aiding in initiation of prompt treatment.

Cerebrotendinous xanthomatosis (a rare lipid storage disorder): a case report.

Science.gov (United States)

Razi, Syed Mohd; Gupta, Abhinav Kumar; Gupta, Deepak Chand; Gutch, Manish; Gupta, Keshav Kumar; Usman, Syeda Iqra

2016-04-19

Cerebrotendinous xanthomatosis is a very rare autosomal recessive lipid storage disorder affecting bile acid biosynthesis. It is manifested by subtle neurological and non-neurological symptoms due to abnormal tissue lipid deposition. Diagnosis is usually delayed but early diagnosis and replacement therapy can prevent devastating neurological sequelae. We present a case of a 25-year-old Asian Indian woman who presented with gait difficulty, fusiform swellings of bilateral tendo-Achilles and infrapatellar tendons, along with history of bilateral cataract surgery 1 year earlier. The diagnosis was made on the basis of clinical, biochemical, imaging, and histopathological analysis and replacement therapy was started. The peculiarity of the present case is the absence of any neurological manifestations which are usually the early clues to the diagnosis of cerebrotendinous xanthomatosis. The present case report emphasizes the fact that early age bilateral cataracts along with bilateral tendo-Achilles xanthomas can be early pointers toward the diagnosis of cerebrotendinous xanthomatosis.

Rapidly Growing Chondroid Syringoma of the External Auditory Canal: Report of a Rare Case

Science.gov (United States)

Vasileiadis, Ioannis; Kapetanakis, Stylianos; Petousis, Aristotelis; Karakostas, Euthimios; Simantirakis, Christos

2011-01-01

Introduction. Chondroid syrinoma of the external auditory canal is an extremely rare benign neoplasm representing the cutaneous counterpart of pleomorphic adenoma of salivary glands. Less than 35 cases have been reported in the international literature. Case Presentation. We report a case of a 34-year-old male in whom a rapidly growing, well-circumscribed tumor arising from the external auditory canal was presented. Otoscopy revealed a smooth, nontender lesion covered by normal skin that almost obstructs the external auditory meatus. MRI was performed to define the extension of the lesion. It confirmed the presence of a 1.5 × 0.8 cm T2 high-signal intensity lesion in the superior and posterior wall of EAC without signs of bone erosion. The patient underwent complete resection of the tumor. The diagnosis was confirmed by histopathologic examination. Conclusion. Although chondroid syringoma is extremely rare, it should always be considered in the differential diagnosis of an aural polyp. Chondroid syringomas are usually asymptomatic, slow-growing, single benign tumors in subcutaneous or intradermal location. In our case, the new information is that this benign tumor could present also as a rapidly growing lesion, arising the suspicion for malignancy. PMID:21941560

Rapidly Growing Chondroid Syringoma of the External Auditory Canal: Report of a Rare Case

Directory of Open Access Journals (Sweden)

Ioannis Vasileiadis

2011-01-01

Full Text Available Introduction. Chondroid syrinoma of the external auditory canal is an extremely rare benign neoplasm representing the cutaneous counterpart of pleomorphic adenoma of salivary glands. Less than 35 cases have been reported in the international literature. Case Presentation. We report a case of a 34-year-old male in whom a rapidly growing, well-circumscribed tumor arising from the external auditory canal was presented. Otoscopy revealed a smooth, nontender lesion covered by normal skin that almost obstructs the external auditory meatus. MRI was performed to define the extension of the lesion. It confirmed the presence of a 1.5×0.8 cm T2 high-signal intensity lesion in the superior and posterior wall of EAC without signs of bone erosion. The patient underwent complete resection of the tumor. The diagnosis was confirmed by histopathologic examination. Conclusion. Although chondroid syringoma is extremely rare, it should always be considered in the differential diagnosis of an aural polyp. Chondroid syringomas are usually asymptomatic, slow-growing, single benign tumors in subcutaneous or intradermal location. In our case, the new information is that this benign tumor could present also as a rapidly growing lesion, arising the suspicion for malignancy.

Radiology illustrated. Uroradiology. 2. ed.

International Nuclear Information System (INIS)

Kim, Seung Hyup

2012-01-01

Uroradiology is an up-to-date, image-oriented reference in the style of a teaching file that has been designed specifically to be of value in clinical practice. All aspects of the imaging of urologic diseases are covered, and case studies illustrate the findings obtained with the relevant imaging modalities in both common and uncommon conditions. Most chapters focus on a particular clinical problem, but normal findings, congenital anomalies, and interventions are also discussed and illustrated. In this second edition, the range and quality of the illustrations have been enhanced, and many schematic drawings have been added to help readers memorize characteristic imaging findings through pattern recognition. The accompanying text is concise and informative. Besides serving as an outstanding aid to differential diagnosis, this book will provide a user-friendly review tool for certification or recertification in radiology. (orig.)

Rare case-series of electrocautery burn following off-pump coronary artery bypass grafting

Science.gov (United States)

Sabzi, Feridoun; Niazi, Mojtaba; Ahmadi, Alireza

2014-01-01

Abstract: With an increasing number of off-pump coronary artery surgery procedures in high-risk patients with coagulopathy, including renal failure, hepatic failure and anticoagulant drug-using patients, the frequency of related complications such as repeated exploration for bleeding is also increasing. The associated co-morbidity and repeated use of electrocautery in postoperative bleeding leaves patients susceptible to electrocautery ulcers. In this case series, rare cases of cautery burn with unique causative mechanisms are described. PMID:23669602

Cervical nodular fasciitis in a 10-year-old girl: a case report of a rare ...

African Journals Online (AJOL)

Thus, as clinical and morphologic characteristics are similar to those of malignant tumours, this entity is often misdiagnosed, with a few cases described in the literature. We present a case of nodular fasciitis involving the retropharynx and hypopharynx of a 10-year-old girl and discuss the importance of considering this rare ...

Laparoscopic-assisted surgical reconstruction of a rare congenital abdominal wall defect in two children misdiagnosed with prune-belly syndrome.

Science.gov (United States)

Fishman, Andrew I; Franco, Israel

2013-08-01

Abdominal wall laxity is typically associated with prune-belly syndrome (PBS). Incomplete forms of PBS have been rarely reported with only the abdominal wall laxity. Herein, we describe a rare congenital abdominal wall defect that has been confused with PBS and illustrate the laparoscopic-assisted surgical technique used for reconstruction. Two boys with symmetrical, bilateral absence or hypoplasia of the internal and external oblique muscles and no genitourinary abnormalities underwent a laparoscopic-assisted abdominal wall reconstruction utilizing the technique previously described by Firlit. Each patient had a Ct scan which confirmed the absence of the oblique muscles. In one patient EMG data confirmed no electrical activity of the obliques. Radiologic evaluation of the urinary tracts revealed no abnormalities. The abdominal wall was plicated utilizing bilateral subcostal incisions. Both patients had excellent cosmetic and functional results with no weakness or bulging of the lateral abdominal wall and improvement of associated symptoms. We believe these two cases and their congenital abdominal wall defects are a rare and often misdiagnosed muscular deficiency separate from PBS. The novel laparoscopic-assisted surgical technique illustrated is feasible and highly successful for these and possible other patients with similar rare congenital abdominal wall defects. Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Two Rare Cases of Hypernatremic Haemorrhagic Encephalopathy in Children.

Directory of Open Access Journals (Sweden)

Dr Shital Turakhia

2016-12-01

Full Text Available Hypernatremia is defined as increased serum sodium concentration more than 145mEq/L. It occurs most commonly in pediatric patients, geriatric patients and patients with debilitated conditions in ICU who suffers from severe water losses. Newborns and toddlers are at high risk as they have poor thirst control and regulatory mechanism. It occurs when patient is suffering from diarrhea and then mismanagement by over treating the patient with sodium containing fluids. CNS complications and radiological findings are rarely recognized and reported. We hereby report cases of two such pediatric patients who were referred to our hospital from primary health care centers with complain of convulsions and diarrhea.

Mycobacterium fortuitum skin infection as a complication of anabolic steroids: a rare case report

Science.gov (United States)

Parampalli, U; Hettiarachchi, G; Ahmed, I

2013-01-01

Mycobacterium fortuitum is a rare cause of recurrent skin abscesses in an immunocompetent person. We report the case of a 37-year-old man presenting with multiple recurrent non-healing skin abscesses. Culture of the abscess wall yielded growth of M fortuitum. In our case, we highlight the association of anabolic steroids with non-tuberculous mycobacterial skin abscesses that fail to resolve despite repeated drainage. PMID:23317715

A very rare spinal cord tumor primary spinal oligodendroglioma: A review of sixty cases in the literature

Directory of Open Access Journals (Sweden)

Askin Esen Hasturk

2017-01-01

Full Text Available Literature review. In this study, we evaluated a case of primary spinal oligodendroglioma (PSO with a rare localization between L3 and S2, and also examined sixty cases in the literature in terms of demographic characteristics, clinical, radiological, and histopathological characteristics, and treatment planning. A case of PSO has been presented, and the relevant literature between 1931 and 2016 was reviewed. A total of 57 papers regarding PSO were found and utilized in this review. The main treatment options include radical surgical excision with neuromonitoring, followed by radiotherapy. Despite these treatment protocols, the relapse rate is high, and treatment does not significantly prolong survival. Oligodendrogliomas are rare among the primary spinal cord tumors. Oligodendrogliomas are predominantly found in the cervical spinal cord, thoracic spinal cord, or junctions during childhood and adulthood. Extension to the sacral region, inferior to the Conus, is very rare. Furthermore, of the sixty cases in the literature, the case we present here is the first to be reported in this particular age group. These localizations usually occur in the pediatric age group and after relapses. While for a limited number of cases the oligodendroglioma initiates in the thoracic region and reaches as far as L2, we encountered a case of an oligodendroglioma within the range of L3 to S2. Clinical findings are observed in accordance with location, and magnetic resonance imaging is the gold standard for diagnosis.

A rare case of Shewanella putrefaciens bacteremia in a patient of road traffic accident

Directory of Open Access Journals (Sweden)

Ritesh Ranjan

2017-01-01

Full Text Available Shewanella putrefaciens rarely causes human infection. These are mostly found in environment and food stuffs. Shewanella are often found in mixed culture. It has been implicated in cellulitis, otitis media, and septicemia. It may be found in respiratory tract, urine, feces, and pleural fluid. There is no definite guideline for therapeutic option. In general, these are susceptible to various antimicrobial agents but are often resistant to penicillin and cephalothin. We report a rare case of bacteremia by S. putrefaciens in a patient of head injury with polytrauma after a road traffic accident.

Plasma cell gingivitis - A rare case related to Colocasia (arbi leaves

Directory of Open Access Journals (Sweden)

Deepika Bali

2012-01-01

Full Text Available Plasma cell gingivitis is an uncommon inflammatory condition of uncertain etiology often flavoured chewing gum, spices, foods, candies, or dentifrices. The diagnosis of plasma cell gingivitis is based on comprehensive history taking, clinical examination, and appropriate diagnostic tests. Here we are presenting a rare case of plasma cell gingivitis caused by consumption of colocasia (arbi leaves. Colocasia is a kind of vegetable, very commonly consumed in the regions of North India.

Retroperitoneal Haematoma in a Patient with Dengue Haemorrhagic Fever: A Rare Case Report.

Science.gov (United States)

Singh, Jasminder; Singh, Harpreet; Sukhija, Gagandeep; Jagota, Ruchi; Bala, Saroj

2016-11-01

Dengue Haemorrhagic Fever (DHF) has diverse manifestations ranging from asymptomatic petechial skin haemorrhages to life threatening cerebral, pulmonary, gastrointestinal and genitourinary haemorrhages. However, the association of spontaneous retroperitoneal haematomas with DHF is not well documented in literature. We report a rare case of spontaneous retroperitoneal haematoma complicating DHF.

An Unusual Erupted Complex Composite Odontoma: A Rare Case

Directory of Open Access Journals (Sweden)

Dawasaz Ali Azhar

2013-01-01

Full Text Available Odontomas are malformations of the dental tissues and may interfere with the eruption of the associated tooth. Complex composite odontoma (CO was described as a distinct entity for the first time by Broca in 1866. This lesion takes place due to the developmental disturbances where the dental components are laid down in a disorganized manner, due to failure of normal morphodifferentiation. Very few cases of erupted complex composite odontomas have been reported in the literature. The case reported here is of an odontoma found in the left mandibular body, associated with an impacted second molar of a 17-year-old Saudi male. Under local anesthesia the odontoma was surgically removed. Histopathological examination confirmed the diagnosis of CO. The impacted second molar which was left in the mandibular body erupted clinically after 6 months. Erupted CO is rarely seen in the mandibular left body. The early diagnosis, followed by a proper treatment at the right time, will result in a favorable prognosis.

Fourth ventricular ependymoma with a distant intraventricular metastasis: Report of a rare case

Directory of Open Access Journals (Sweden)

Sudheer Ambekar

2013-01-01

Full Text Available Ependymoma is one of the uncommon tumors of the central nervous system (CNS in the adult age group. These tumors have a distinct propensity for metastasis, both within and outside the CNS. However, dissemination at the time of first presentation and retrograde dissemination of the tumor is rare. We report the case of a patient with fourth ventricular anaplastic ependymoma who presented with left lateral ventricular metastasis which was anatomically different from the primary tumor. We describe the clinic-pathological detail of the patient and discuss the probable pathophysiological basis for this rare presentation and its significance in management of the patient.

Primary Ewing's Sarcoma of the Temporal Bone: A Rare Case Report and Literature Review.

Science.gov (United States)

Gupta, Divya; Gulati, Achal; Purnima

2017-09-01

Ewing's sarcoma is a malignant, round cell tumor arising from the bones and primarily affecting children and adolescent, accounting for 3 % of all childhood malignancies. Although the long bones and the trunk are typically affected, rare cases of it involving isolated bones throughout the body have been reported. Involvement of the skull bones is rare, constituting 1-6 % of the total Ewing's sarcoma cases but those affecting the cranial bones are rarer still, constituting only 1 %. We describe an 8 months old infant having Ewing sarcoma, of the petrous and mastoid parts of temporal bone along with the occipital bone, whose clinical presentation mimicked mastoiditis with facial nerve palsy. We discuss the clinical and therapeutic course of an extensive primary Ewing sarcoma of the temporal bone, which was treated without performing surgery and review this entity's literature in detail.

Rare malposition following left jugular vein catheterization: Case reports and a literature review.

Science.gov (United States)

Wang, Lin; Liu, Zhangsuo; Wang, Changan; Liu, Dongwei; Yuan, Yiqiang

2015-01-01

The number of patients requiring chronic hemodialysis via a tunneled cuffed catheter is increasing. The right internal jugular vein (IJV) is generally the preferred site of percutaneous insertion. In certain situations, for example, in patients with a history of multiple access failures, catheterization of the left IJV is an important option. In this report, we present two rare cases of catheter malposition after left IJV catheterization; catheter adjustments, with the help of chest radiographs, resulted in a positive outcome in both cases. These cases exemplify the difficulties associated with left IJV catheterization, of which there are few reports in the published literature.

Calcifying epithelial odontogenic tumor, a rare presentation in children: Two case reports

Directory of Open Access Journals (Sweden)

Susant Mohanty

2014-01-01

Full Text Available Calcifying epithelial odontogenic tumor (CEOT is a rare and benign odontogenic neoplasm that affects the jaws. It is certainly an atypical instance to find this tumor in children. Here, we present two case reports of CEOT presenting in mandible of a 12- and 13-year-old female child, respectively. CEOT have been reported to show features of malignant transformation also.

Extramammary Paget ’s disease Of Glans Penis: A Rare Case Report

LENUS (Irish Health Repository)

Inder, M S

2018-06-01

We present the case of an 83-year-old man with Extramammary Paget’s disease (EMPD) of the penis. He underwent a total penectomy and histopathology confirms the association of underlying invasive high grade urothelial carcinoma. Penile EMPD is rare and can be misinterpreted for benign skin conditions. A high index of suspicion is required for correct diagnosis and appropriate treatment.

EXTENSIVE RADICULAR CYST OF THE MANDIBLE: A RARE CASE REPORT

Directory of Open Access Journals (Sweden)

Gokul VENKATESHWAR

2013-03-01

Full Text Available The radicular cyst is the most common inflammatory odontogenic cystic lesion of the jaws. It usually originates as a sequel to a periapical inflammatory process, following chemical, physical or bacterial injury. Due to its chronic etiology, the cyst usually appears towards the later stage of life. It has a male sex predilection, with the maxillary anterior region as the most common site of involvement. This article reports a rare case of a large radicular cyst in the mandible, its management and follow up along one year.

A RARE CASE OF HUGE CERVICAL FIBROID

Directory of Open Access Journals (Sweden)

Tushar Tatyaba Palve

2017-07-01

Full Text Available PRESENTATION OF CASE Leiomyomas are the most common tumor of uterus and the female pelvis. It is impossible to determine true incidence, all though frequently quoted incidence of 50% seems reasonable at post mortem examination1 . However only 1- 2% of it are confined to cervix. Cervical leiomyomata are mostly single and are subserous or interstitial in origin. Generally these tumours presents with retention of urine, constipation, sensation of something coming out of vagina, & rarely at times present with abdominal mass which may mimic ovarian cancer. 47 year old unmarried nulligravida with complaints of severe dysmenorrhagia since one year and lump in abdomen which has grown over period of one year. There was no bowel and bladder complaints, On physical examination pallor present, on abdominal examination 26-28 week firm solid mass arising from pelvis with restricted mobility was present, Clinically no ascites, patient was investigated on OPD basis.

Chylous ascites associated with chylothorax; a rare sequela of penetrating abdominal trauma: a case report

Directory of Open Access Journals (Sweden)

Plummer Joseph M

2007-11-01

Full Text Available Abstract We present the case of a patient with the rare combination of chylous ascites and chylothorax resulting from penetrating abdominal injury. This patient was successfully managed with total parenteral nutrition. This case report is used to highlight the clinical features and management options of this uncommon but challenging clinical problem.

A Rare Cause of Fatty Liver and Elevated Aminotransferase Levels: Chanarin-Dorfman Syndrome: A Case Report

Directory of Open Access Journals (Sweden)

Özdal Ersoy

2011-01-01

Full Text Available Chanarin-Dorfman syndrome is a rare, inherited metabolic disorder of neutral lipid storage characterized by ichthyosis, lipid vacuoles in leukocytes, and involvement of several internal organs, mostly the liver. Since the initial case was reported by Dorfman in 1974, nearly 50 cases have been reported, and the majority were from Middle East countries. Here, we report a 20-year-old patient with ichthyosis from Turkey, diagnosed as Chanarin-Dorfman syndrome presented with asypmtomatic elevated transaminases and hepatosteatosis, and also briefly review the updated clinical implications and management of this rarely seen syndrome. Prompt diagnosis of this syndrome avoids further unnecessary investigations in patients with ichthyosis

Submucosal lipoma of the sigmoid colon as a rare cause of mucoid diarrhea: a case report.

Science.gov (United States)

Dassanayake, S U B; Dinamithra, N P; Nawarathne, N M M

2016-01-20

Symptomatic presentations of colonic lipomas are very rare in clinical practice, and may mimic colonic malignancy. The likelihood of presenting symptoms has been shown to depend on the size of the lesion. We describe the case of a 72-year-old Sinhalese man presenting with worsening mucoid diarrhea who was subsequently diagnosed to have a lipoma of the sigmoid colon. His disease was successfully managed with endoscopic resection. Confidently establishing the rare diagnosis of a colonic lipoma usually requires a combination of endoscopic, radiological, and histological evaluation, and is therefore very challenging. With the advancement of endoscopic procedures, endoscopic resection is widely practiced as the definitive management of these cases.

Large Complex Odontoma of Mandible in a Young Boy: A Rare and Unusual Case Report

Directory of Open Access Journals (Sweden)

G. Siva Prasad Reddy

2014-01-01

Full Text Available Odontomas are the most common odontogenic tumors. They are broadly classified in to Compound Odontoma and Complex Odontoma. Among them complex odontoma is a rare tumor. Occasionally this tumor becomes large, causing expansion of bone followed by facial asymmetry. Otherwise these tumors are asymptomatic and are generally diagnosed on radiographic examination. We report a rare case of complex odontoma of mandible in a young boy. The tumor was treated by surgical excision under general anesthesia.

Spontaneous Superior Mesenteric Artery Branch Pseudoaneurysm: A Rare Case Report

Directory of Open Access Journals (Sweden)

Mina Guirgis

Full Text Available : Background: Visceral arterial pseudoaneurysms (VAPAs are rare vascular entities with serious consequences. Traditionally, they are associated with trauma, infection, and inflammatory disease, or they can arise as a post-operative complication. Report: An 87 year old man presented with abdominal pain and was found to have a spontaneous VAPA on a computed tomography angiogram. Serial imaging 4 months previously had demonstrated no aneurysm. Between scans, warfarin was changed to apixaban for aortic valve replacement, but he had no other changes to any other medications. He required urgent endovascular coiling of the pseudoaneurysm, with satisfactory recovery and outcome. Discussion: VAPAs are extremely rare, with splenic artery VAPAs the most commonly reported. Regardless, fewer than 250 cases of splenic artery pseudoaneurysm have been reported. Superior mesenteric artery (SMA pseudoaneurysms are the rarest type of VAPAs. Early identification and urgent treatment are warranted because of the associated high mortality risk, with a 50% risk of rupture in any given VAPA. Treatment options range from open operation to endoscopic and endovascular procedures. Apixaban has been proposed to contribute to pseudoaneurysm formation by slow and continuous bleeding that results in the formation of the pseudoaneurysm. Conclusions: Spontaneous VAPAs are extremely rare and this is the first time a VAPA has been associated with the novel oral anticoagulant “apixaban”. Urgent management of any VAPAs is important because of the high risk of rupture and potential life threatening haemorrhage. Keywords: Visceral arterial pseudoaneurysm, Superior mesenteric artery, Apixaban, Coils

Semimembranosus muscle herniation: a rare case with emphasis on muscle biomechanics

Energy Technology Data Exchange (ETDEWEB)

Naffaa, Lena [American University of Beirut, Department of Diagnostic Radiology, P.O. Box 11-0236, Riad El-Solh, Beirut (Lebanon); Moukaddam, Hicham [Saint Rita Medical Center, Lima, OH (United States); Samim, Mohammad [New York University, Department of Radiology, Hospital for Joint Disease, New York, NY (United States); Lemieux, Aaron [University of California, San Diego School of Medicine, La Jolla, CA (United States); Smitaman, Edward [University of California, San Diego, Teleradiology and Education Center, San Diego, CA (United States)

2017-03-15

Muscle herniations are rare and most reported cases involve muscles of the lower leg. We use a case of muscle herniation involving the semimembranosus muscle, presenting as a painful mass in an adolescent male after an unspecified American football injury, to highlight a simple concept of muscle biomechanics as it pertains to muscle hernia(s): decreased traction upon muscle fibers can increase conspicuity of muscle herniation(s) - this allows a better understanding of the apt provocative maneuvers to employ, during dynamic ultrasound or magnetic resonance imaging, in order to maximize diagnostic yield and, thereby, limit patient morbidity related to any muscle herniation. Our patient subsequently underwent successful decompressive fasciotomy and has since returned to his normal daily activities. (orig.)

Semimembranosus muscle herniation: a rare case with emphasis on muscle biomechanics

International Nuclear Information System (INIS)

Naffaa, Lena; Moukaddam, Hicham; Samim, Mohammad; Lemieux, Aaron; Smitaman, Edward

2017-01-01

Muscle herniations are rare and most reported cases involve muscles of the lower leg. We use a case of muscle herniation involving the semimembranosus muscle, presenting as a painful mass in an adolescent male after an unspecified American football injury, to highlight a simple concept of muscle biomechanics as it pertains to muscle hernia(s): decreased traction upon muscle fibers can increase conspicuity of muscle herniation(s) - this allows a better understanding of the apt provocative maneuvers to employ, during dynamic ultrasound or magnetic resonance imaging, in order to maximize diagnostic yield and, thereby, limit patient morbidity related to any muscle herniation. Our patient subsequently underwent successful decompressive fasciotomy and has since returned to his normal daily activities. (orig.)

Capnocytophaga canimorsus: a rare case of conservatively treated prosthetic valve endocarditis.

Science.gov (United States)

Jalava-Karvinen, Päivi; Grönroos, Juha O; Tuunanen, Helena; Kemppainen, Jukka; Oksi, Jarmo; Hohenthal, Ulla

2018-05-01

We describe a rare case of prosthetic valve endocarditis caused by the canine bacterium Capnocytophaga canimorsus in a male aged 73 years. The diagnosis of infective endocarditis was unequivocal, as it blood cultures were positive for C. canimorsus and vegetations were detected on transesophageal echocardiography; the modified Duke criteria were fulfilled. PET-CT showed intense 18 F-FDG uptake of the prosthetic valve area. The patient was treated with antibiotics alone (no surgery), and is now on life-long suppressive antibiotic therapy. To our knowledge, this is the third reported case of prosthetic valve endocarditis caused by C. canimorsus and the first one to have been treated conservatively. © 2018 APMIS. Published by John Wiley & Sons Ltd.

A Rare Case of Radiologically Not Distinguishable Coexistent Meningioma and Vestibular Schwannoma in the Cerebellopontine Angle – Case Report and Literature Review

Directory of Open Access Journals (Sweden)

Juergen Grauvogel

2010-08-01

Full Text Available Background: The simultaneous occurrence of cerebellopontine angle (CPA meningioma and vestibular schwannoma (VS in the absence of neurofibromatosis type 2 or history of irradiation is very rare. We report a case with coexistent CPA meningioma and VS, which were radiologically not distinguishable in preoperative imaging. Case Description: A 46-year-old female presented with acute hearing loss, tinnitus and gait ataxia. Otorhinolaryngological diagnostic workup and imaging studies showed an intra- and extrameatal homogenous contrast enhancing lesion. The neuroradiological diagnosis was VS. The patient was operated via the retrosigmoid approach. Intraoperatively two distinct tumors were found: a small, mainly intrameatally located VS and a larger meningioma originating from the dura of the petrous bone. Both tumors were completely microsurgically removed. The patient experienced no new neurological deficit after surgery; particularly facial nerve function was completely preserved. Histopathological examination revealed a fibromatous meningioma and a VS, respectively. Conclusions: The coincidental occurrence of CPA meningioma and VS is very rare. Careful interpretation of imaging studies before surgery is crucial. Even such rare cases should be kept in mind when discussing the therapeutic options with the patient. More studies are needed for a better understanding of mechanisms leading to multiple tumor growth.

Ultrasound and MR-imaging in preoperative evaluation of two rare cases of scar endometriosis

OpenAIRE

Pados, George; Tympanidis, John; Zafrakas, Menelaos; Athanatos, Dimitrios; Bontis, John N

2008-01-01

Scar or incisional endometriosis is a rare, often misdiagnosed, pathologic condition of the abdominal wall. Two cases of incisional endometriosis are presented. Both patients presented with atypical cyclic pain and palpable nodules on scars of previous cesarean sections. In both cases, the mass was totally excised, after accurate preoperative evaluation with 2-D ultrasound, power Doppler and MRI. Microscopic examination confirmed the preoperatively presumed diagnosis of cutaneous endometriosi...

A rare case of quadrigeminal plate lipoma presenting with the sixth cranial nerve palsy

Institute of Scientific and Technical Information of China (English)

Bipin Kumar Chaurasia; Tolga Dundar; Narendra Shalike; Silak Ram Chaudhary; Shamsul Alam; Dhiman Chowdhory; Kanak Kanti Barua; Ranjit Kumar Chaurasiya; Raushan Kumar Chaurasia; Ramesh Kumar Chaurasia

2017-01-01

Intracranial lipomas are rare benign tumour that is slow growing, generally asymptomatic, most frequently located in the midline areas and are usually an incidental finding on imaging and therefore cases are not frequently reported. This study reports a case of a patient with quadrigeminal plate lipoma presenting with obstructive hydrocephalous and the 6th cranial nerve palsy that was successfully treated with ventriculo-peritoneal shunting without addressing the lesion.

Recurrent diabetic muscle infarction, a rare complication of diabetes: a case report

Directory of Open Access Journals (Sweden)

Tariq Bhat

2017-01-01

Full Text Available Diabetic muscle infarction is a rare complication of diabetes mellitus that presents as a localized, exquisitely painful swelling and limited range of motion of the involved extremity. The onset is usually acute, persists for several weeks and resolves spontaneously over several weeks to months without the need for intervention. However, as diabetes mellitus is an immunocompromised state and any painful swelling in the limbs is often taken as infectious in aetiology, the patient is inadvertently investigated with invasive procedures and is started on unnecessary antibiotics, adding to the burden of management. Keeping in view the low threshold for starting antibiotics in painful limb swelling in diabetes mellitus in our setting, we hereby describe a case of recurrent painful diabetic muscle infarction, first involving the right upper and later the right lower limb, managed with physical rest and analgesics. This case emphasizes that the treating physician keep this rare complication of diabetes mellitus in consideration in the respective clinical scenario and adopt a less aggressive (a noninvasive method like ultrasound rather than a more aggressive (an invasive method like muscle biopsy approach in diagnosis and take a similar approach towards management.

A Rare Case of Spontaneous Pneumocephalus Associated with Nontraumatic Cerebrospinal Fluid Leak

Directory of Open Access Journals (Sweden)

Murad Baba

2016-01-01

Full Text Available Introduction. Spontaneous nontraumatic pneumocephalus (PNC and cerebrospinal fluid (CSF leaks are both very uncommon conditions. We report a rare case of spontaneous pneumocephalus associated with CSF leak secondary to right sphenoid sinus bony defect without history of trauma. Case Description. 51-year-old Hispanic female with past medical history of hypertension and idiopathic intracranial hypertension (Pseudotumor Cerebri presented to the emergency room complaining of headache and clear discharge from the right nostril. Physical examination was significant for right frontal sinus tenderness and clear discharge from right nostril. Computed Tomography (CT scan of the brain showed moderate amount of extra-axial air within the right cerebral hemisphere indicative of pneumocephalus. CT scan of facial bones showed bony defect along the right sphenoid sinus with abnormal CSF collection. The patient was started on intravenous antibiotics for meningitis prophylaxis and subsequently underwent transsphenoidal repair of cerebrospinal fluid leak with abdominal fat graft. CSF rhinorrhea stopped completely after the surgery with near complete resolution of pneumocephalus before discharge. Conclusions. Early identification of pneumocephalus and surgical intervention can help decrease the morbidity and avoid possible complications. Idiopathic intracranial hypertension, although rare, can lead to CSF leak and pneumocepahlus.

A rare case of short stature: Say Meyer syndrome.

Science.gov (United States)

Karthik, T S; Prasad, N Rajendra; Rani, P Radha; Maheshwari, Rushikesh; Reddy, P Amaresh; Chakradhar, B V S; Menon, Bindu

2013-10-01

Say Meyer syndrome is rare X linked condition characterized by developmental delay, short stature and metopic suture synostosis. We are reporting a case of Say Meyer syndrome presented to our hospital for short stature and developmental delay at age 3½ years. A 3½-year-old boy presented to our hospital for decreased growth velocity from the age of 1 year. History revealed the boy had a birth weight of 2.3 kg, had an episode of seizures in the neonatal period. He was born to non-consanguineous marriage. He had global developmental delay and there was a lack of bowel and bladder control. History did not reveal any hearing or visual impairment. No history of any chronic systemic illnesses. Magnetic resonance imaging (MRI) brain revealed mild diffuse frontotemporal atrophy with multiple irregular gliotic areas in bilateral frontal lobes. Diffuse white matter volume loss in bilateral cerebral hemispheres. Diffuse thinning of corpus callosum. Diffuse periventricular hyper intensity on T2W and fluid attenuated inversion recovery sequences. Say Meyer syndrome is rare X linked condition characterized by developmental delay, short stature and metopic suture synostosis. Characteristic MRI brain findings include diffuse frontotemporal atrophy with multiple gliotic areas in frontal lobes. Diffuse white matter volume loss in bilateral cerebral hemispheres.

Rare case of malignant craniopharyngioma reactive to adjunctive stereotactic radiotherapy and chemotherapy; Case report and review.

Science.gov (United States)

Nomura, Shunsunke; Aihara, Yasuo; Amano, Kosaku; Eguchi, Seiichiro; Chiba, Kentaro; Komori, Takashi; Kawamata, Takakazu

2018-06-19

Malignant craniopharyngioma or anaplastic craniopharyngioma was first reported in 1987 by Akachi. It has a malignant clinical and histological feature; remarkably rapid progression, atypical pathology like squamous cell carcinoma and poor prognosis. To date seventeen cases of malignant craniopharyngioma have been reported and of these cases, most were of secondary malignant tumor in nature. With respect to traditional benign craniopharyngioma, adjunctive treatment after gross total removal is not necessary, but in the case of malignant types of the tumor, adjunctive treatment is important. This paper presents the first case of malignant craniopharyngioma reactive to adjunctive Gamma knife stereotactic radiosurgery and chemotherapy. Malignant craniopharyngioma is very rare, and we report Gamma knife stereotactic radiosurgery and chemotherapy (Carboplatine and etoposide chemotherapy), as well as Temozolomide chemotherapy were effective and could control progression of the tumor temporarily. Since adjunctive Gamma knife stereotactic radiosurgery and chemotherapy of malignant craniopharyngioma cases affects follow-up strategies, we propose supporting the need to a revision to the WHO classification regarding malignancy evaluation of craniopharyngioma. Copyright © 2018 Elsevier Inc. All rights reserved.

A rare case report of grade III gingival enlargement associated with chronic periodontitis: Comparison of two treatment techniques.

Science.gov (United States)

Pant, Vandana A; Pandey, Suraj

2015-01-01

Idiopathic gingival enlargement (GE) is a rare entity characterized by massive enlargement of the gingiva. It may be associated with other diseases/conditions characterizing a syndrome, but rarely documented in literature occurring along with chronic periodontitis. This case report describes a rare case of long standing massive GE in a systemically healthy, nonsyndromic young female involving both the arches, thereby posing a diagnostic dilemma. Furthermore, in this case, we compared two surgical approaches, that is, scalpel and electrosurgery for the convenience as well as the postoperative comfort of the patient. Quadrants 1 and 3 were treated by ledge and wedge technique using scalpel and blade while quadrants 2 and 4 were treated by electrosurgery. The patient was followed postoperatively up to 1-year. The massive GE subsided without recurrence and patient was completely satisfied with the treatment, though better compliance was observed at the site treated by conventional scalpel and blade technique.

Case Report: Sciatic nerve schwannoma - a rare cause of sciatica [version 1; referees: 2 approved

Directory of Open Access Journals (Sweden)

Sunil Munakomi

2017-03-01

Full Text Available Herein we report a rare case of a sciatic nerve schwannoma causing sciatica in a 69-year-old female. Sciatic nerve schwannoma is a rare entity. It should always be considered as a possible cause of sciatica in patients that present with symptoms of sciatica with no prolapsed disc in the lumbar spine and a negative crossed straight leg raise test. Timely diagnosis and complete excision of the lesion leads to complete resolution of the symptoms of such patients.

Neuroblastoma in early childhood: A rare case report and review of literature

Directory of Open Access Journals (Sweden)

Ritesh R Kalaskar

2016-01-01

Full Text Available Neuroblastoma is an extremely rare pediatric neoplasm whose prognosis becomes poor and poor as the age advances. It can be sporadic or nonfamilial in origin. It is primarily a tumor of abdominal origin from where it metastasis to lymph nodes, liver, intracranial and orbital sites, and central nervous system. There is no standard dental treatment protocol for the management of neuroblastoma due to its poor survival rate and rarity. However, dental treatment may follow the protocol of preventive and restorative. Surgicals should be performed under supervision as it may trigger metastasis. We report a rare case of neuroblastoma in a 3-year-old child presenting classical oral manifestations such as bilateral palatal swelling, rolled border ulcer on the posterior part of hard palate adjacent to primary molars, and bilateral proptosis.

Pancreatic metastasis from invasive pleomorphic lobular carcinoma of the breast: a rare case report.

Science.gov (United States)

Sun, Xiangjie; Zuo, Ke; Huang, Dan; Yu, Baohua; Cheng, Yufan; Yang, Wentao

2017-07-11

Invasive pleomorphic lobular carcinoma (PLC) is an aggressive subtype of invasive lobular carcinoma of the breast, which has its own histopathological and biological features. The metastatic patterns for PLC are distinct from those of invasive ductal carcinoma. In addition, pancreatic metastasis from PLC is extremely rare. We report a rare case of a 48-year-old woman presenting with clinical gastrointestinal symptoms and pancreatic metastasis of PLC. The pancreatic tumor was composed of pleomorphic tumor cells arranged in the form of solid sheets and nests and as single files, with frequent mitotic figures, nucleolar prominence, high nuclear to cytoplasmic ratio and loss of cohesion. The malignant cells were positive for p120 (cytoplasmic) and GATA3 and negative for estrogen receptor, progesterone receptor, human epidermal growth factor receptor 2, E-cadherin, gross cystic disease fluid protein 15 and mammaglobin, which indicated a lobular carcinoma phenotype of the breast. To the best of our knowledge, this is one of the few reported cases in the literature of pancreatic metastasis of invasive lobular carcinoma of the breast, of which the definitive diagnosis was obtained only after surgery. Rare metastasis sites should be considered, particularly, when a patient has a medical history of PLC.

Successful treatment of rare-earth magnet ingestion via minimally invasive techniques: a case series.

Science.gov (United States)

Kosut, Jessica S; Johnson, Sidney M; King, Jeremy L; Garnett, Gwendolyn; Woo, Russell K

2013-04-01

Cases of rare-earth magnet ingestions have been increasingly reported in the literature. However, these descriptions have focused on the severity of the injuries, rather than the clinical presentation and/or therapeutic approach. We report a series of eight children, ranging in age from 2 to 10 years, who ingested powerful rare-earth magnets. The rare-earth magnets were marketed in 2009 under the trade name Buckyballs(®) (Maxfield & Oberton, New York, NY). They are about 5 mm in size, spherical, and brightly colored, making them appealing for young children to play with and place in their mouths. Three children presented within hours of ingestion, and the magnets were successfully removed via endoscopy in two, whereas the third child required laparoscopy. No fistulas were found in these children. A fourth child presented 2 days after ingestion with evidence of bowel wall erosion, but without fistula formation; the magnets were removed via laparoscopy. A fifth child ingested nine magnets in a ring formation, which were removed via colonoscopy without evidence of injury or fistula formation. The three remaining children presented late (5-8 days after ingestion) and were found to have associated fistulas. They were treated successfully with a combination of endoscopy and laparoscopy with fluoroscopy. None of the children in our series required an open surgical procedure. All children were discharged home without complications. This case series highlights the potential dangers of rare-earth magnet ingestion in children. Our experience suggests that prompt intervention using minimally invasive approaches can lead to successful outcomes.

Adenocarcinoma of the urinary bladder, mesonephroid type: a rare case

Directory of Open Access Journals (Sweden)

Mahmoud Abbas

2013-02-01

Full Text Available Primary adenocarcinoma of the urinary bladder is a rare disease. It occurs in 0.5-2% of all bladder cancers and is discussed as the malignant counterpart of nephrogenic adenomas. We report a 46-year-old white female presented with gross hematuria for clinical examination. Histopathology revealed pT2, Pn1, L1, G2 adenocarcinoma of the bladder and carcinoma in situ according to the TNM classification. Computed tomography scan diagnostic was unremarkable. Patients with adenocarcinoma of the urinary bladder should be treated vigorously and without time delay. Only 7 cases of adenocarcinoma in the urinary bladder (mesonephroid have been described until now. We present a case of clear cell adenocarcinoma of the urinary bladder, mesonephroid type that early diagnosed and till now 3 months after the cystectomy without symptoms and without complications.

A rare pediatric case of grossly dilated ureter presenting as abdominal mass

International Nuclear Information System (INIS)

Srivastava, Madhur Kumar; Govindarajan, Krishna Kumar; Chakkalakkoombil, Sunitha Vellathussery; Halanaik, Dhanapathi

2016-01-01

Renal masses account for 55% of cases presenting as palpable abdominal mass in children.[1] An eight year male presented with palpable abdominal mass and pain. The patient underwent renal dynamic scan, which raised possibility of left duplex kidney with non-functioning moiety, as the size of left kidney was smaller than seen on Ultrasonography (USG). Magnetic resonance (MR)urography confirmed the findings with patient undergoing left hemi-nephrectomy and is doing well. In case of discrepancy in size of kidney on USG and renal scan, duplex kidney should be considered as differential, other causes being, renal cyst, benign/malignant mass and renal calculi. Gross hydro-ureter presenting as palpable abdominal mass is very rare with few reported cases.[234

A rare case of minimal deviation adenocarcinoma of the uterine cervix in a renal transplant recipient.

LENUS (Irish Health Repository)

Fanning, D M

2009-02-03

INTRODUCTION: We report the first described case of minimal deviation adenocarcinoma of the uterine cervix in the setting of a female renal cadaveric transplant recipient. MATERIALS AND METHODS: A retrospective review of this clinical case was performed. CONCLUSION: This rare cancer represents only about 1% of all cervical adenocarcinoma.

A rare case of minimal deviation adenocarcinoma of the uterine cervix in a renal transplant recipient.

LENUS (Irish Health Repository)

Fanning, D M

2012-02-01

INTRODUCTION: We report the first described case of minimal deviation adenocarcinoma of the uterine cervix in the setting of a female renal cadaveric transplant recipient. MATERIALS AND METHODS: A retrospective review of this clinical case was performed. CONCLUSION: This rare cancer represents only about 1% of all cervical adenocarcinoma.

An Unusual Endovascular Therapeutic Approach for a Rare Case of May-Thurner Syndrome.

Science.gov (United States)

DaSilva-DeAbreu, Adrian; Masha, Luke; Peerbhai, Shareez

2017-03-06

BACKGROUND The etiology of deep venous thrombosis (DVT) may pose a significant diagnostic challenge because truly reversible causes of DVT are rare. In this regard, known pelvic anatomic abnormalities such as aortic and iliac aneurysms should be seriously considered as a complicating factor in patients presenting with acute DVT so as not to miss a potentially curable etiology of May-Thurner syndrome (MTS). CASE REPORT We report the case of a 69-year-old man with a known abdominal aortic aneurysm and bilateral iliac artery aneurysms who presented with an acute DVT. A computed tomography scan of the abdomen and pelvis showed increased dilation of his aneurysmal disease with new resultant compression of the left iliac vein representing acquired MTS. The patient underwent endovascular aneurysm repair of the infra-renal abdominal aortic aneurysm and right common iliac artery aneurysm with a Gore Excluder endoprosthesis in lieu of venous stenting, with resolution of symptoms. CONCLUSIONS Infra-renal aortic and iliac aneurysms causing MTS are extremely rare, and patients at risk for MTS through these mechanisms do not fit the classical demographics associated with this syndrome. Furthermore, this is the first case described in which MTS was treated by addressing the aneurysm through an endoprosthetic approach instead of venous stenting, which is the conventional intervention for MTS.

Radiation-induced spindle cell sarcoma: A rare case report

Directory of Open Access Journals (Sweden)

Khan Mubeen

2009-01-01

Full Text Available Ionizing radiation has been known to induce malignant transformation in human beings. Radiation-induced sarcomas are a late sequel of radiation therapy. Most sarcomas have been reported to occur after exposure to a radiation dose of 55 Gray (Gy and above, with a dose ranging from 16 to 112 Gys. Spindle cell sarcomas, arising after radiotherapy given to treat the carcinoma of head and neck region is a very uncommon sequel. This is a rare case report of spindle cell sarcoma of left maxilla, in a 24-year-old male, occurring as a late complication of radiotherapy with Cobalt-60 given for the treatment of retinoblastoma of the left eye 21 years back.

A rare case of trichotillomania with antisocial personality disorder

Directory of Open Access Journals (Sweden)

Priti Singh

2015-01-01

Full Text Available Trichotillomania (TTM is characterised by recurrent and irresistible urge to pull out one’s own body hair. It is often associated with trichorrhizophagia in which there is a habit to eat the roots of pulled out hairs. It can also present with many comorbid psychiatric problems including personality disorders. High rates of comorbid mood, anxiety, and substance use disorders have been detected in patients of TTM. The lifetime prevalence of comorbid personality disorders has been much less extensively studied. We present a rare case of 28-year-old male having TTM with antisocial personality disorder and discuss difficult management issues with this comorbidity. Our patient improved with a combination of fluoxetine and sodium valproate.

The Insignificance of Thresholds in Environmental Impact Assessment: An Illustrative Case Study in Canada

Science.gov (United States)

Murray, Cathryn Clarke; Wong, Janson; Singh, Gerald G.; Mach, Megan; Lerner, Jackie; Ranieri, Bernardo; Peterson St-Laurent, Guillaume; Guimaraes, Alice; Chan, Kai M. A.

2018-06-01

Environmental assessment is the process that decision-makers rely on to predict, evaluate, and prevent biophysical, social, and economic impacts of potential project developments. The determination of significance in environmental assessment is central to environmental management in many nations. We reviewed ten recent environmental impact assessments from British Columbia, Canada and systematically reviewed and scored significance determination and the approaches used by assessors, the use of thresholds in significance determination, threshold exceedances, and the outcomes. Findings of significant impacts were exceedingly rare and practitioners used a combination of significance determination approaches, most commonly relying upon reasoned argumentation. Quantitative thresholds were rarely employed, with less than 10% of the valued components evaluated using thresholds. Even where quantitative thresholds for significance were exceeded, in every case practitioners used a variety of rationales to demote negative impacts to non-significance. These reasons include combinations of scale (temporal and spatial) of impacts, an already exceeded baseline, model uncertainty and/or substituting less stringent thresholds. Governments and agencies can better protect resources by requiring clear and defensible significance determinations, by making government-defined thresholds legally enforceable and accountable, and by requiring or encouraging significance determination through inclusive and collaborative approaches.

Carotid cavernous fistula after elective carotid endarterectomy: Case report

Directory of Open Access Journals (Sweden)

Andres Asser

2014-12-01

This is a case to illustrate a rare complication of carotid artery surgery. The patient had atherosclerotic vessel damage of ICA visible on earlier CT scans. This combined with abrupt increase of transmural pressure due to the revascularization procedure could possibly lead to arterial wall rupture and fistula formation.

A Rare Case Report of a Child Coexistence Thyroglossal Cyst and Second Branchial Cleft Fistulae.

Science.gov (United States)

Mahdoufi, Rachid; Barhmi, Ismail; Tazi, Nabil; Rouadi, Sami; Abada, Reda; Roubal, Mohamed; Mahtar, Mohamed

2017-06-01

Thyroglossal duct cysts followed by branchial cleft anomalies are the most common congenital neck masses encountered in practice, second branchial cleft cysts and sinuses are the most common type (LaRiviere and Waldhausen in Surg Clin North Am 92(3):583-597, 2012). Although both abnormalities are common individually, but rarely seen associated in same patient as described in our case. Congenitalcervical anomalies are important to consider in the differential of head and neck masses in children and adults. These lesions can present as palpable cystic masses, infected masses, draining sinuses, or fistulae. Thyroglossal duct cysts are most common, followed by branchial cleft anomalies. A synchronous presentation of both type of cyst and fistula in a same child patient is very rare with no such cases reported in literature till date.

Primary chondrosarcoma of breast - cytology with histopathological correlation: A rare case report with review of literature

Directory of Open Access Journals (Sweden)

Sankappa P. Sinhasan

2014-01-01

Full Text Available Malignant mesenchymal tumors of the breast other than angiosarcoma are extremely rare and comprise <0.5% of breast tumors. Primary chondrosarcoma of the breast is an extremely rare entity and only 10 cases are reported as single case reports in literature until date. A diagnosis of primary mammary sarcoma can be established only after excluding metaplastic carcinomas and malignant phyllodes by extensive sampling for evidence of in situ or invasive carcinoma. Here, we report a primary chondrosarcoma of breast in a 55-year-old lady diagnosed precisely on fine-needle aspiration cytology and confirmed by histopatholigcal examination after total mastectomy. We emphasize on diagnostic difficulties encountered in cytology smears and discuss differential diagnoses.

Pleomorphic Hyalinizing Angiectatic Tumour: A Rare Case Report and Discussion of Differential Diagnosis.

Science.gov (United States)

Chalmeti, Ambica; Arakeri, Surekha U; Javalgi, Anita P; Goyal, Shefali

2017-08-01

Pleomorphic Hyalinizing Angiectatic Tumour (PHAT) is one of the rare soft tissue tumour which is non-metastasizing. The origin of this tumour is yet uncertain. It occurs in adults as a slow growing subcutaneous mass mimicking clinically and histologically to various benign and malignant soft tissue tumours such as schwannoma, haemangioma and malignant fibrous histiocytoma. The microscopic features of this tumour include clusters of ectatic, fibrin containing, hyalinized blood vessels with pleomorphic and spindle shaped tumour cells showing intranuclear inclusions, stromal haemosiderin pigment and a variable inflammatory infiltrate. Despite marked pleomorphism, the lesion behaves as a low grade neoplasm, with frequent recurrences, but no metastases. The incidence of this tumour is very rare with less than 100 cases being published. Hence, awareness of this entity is must for proper management of the patient and to avoid misdiagnosis of the lesion. We report a case of pleomorphic hyalinizing angiectatic tumour in a 50-year-old man who presented with a slow growing mass in the left calf region since two years.

Lymphoepithelial carcinoma: a case report of a rare tumor of the larynx.

Science.gov (United States)

Hammas, Nawal; Benmansour, Najib; El Alami El Amine, Mohamed Nour-Dine; Chbani, Laila; El Fatemi, Hind

2017-01-01

Lymphoepithelial carcinoma is a tumor mostly diagnosed in the nasopharynx, but it has also been described in a variety of nonnasopharyngeal sites. It is extremely rare in the larynx and should be distinguished from squamous cell carcinoma. Therefore, it must be known by clinicians, pathologists and oncologists. In this case report, we discuss its etiopathogeny, its epidemiological, clinical, pathological and therapeutic aspects, and its outcome. An 81-year-old Morrocan man, smoker for 40 years, presented with a 1 year history of dysphonia, dyspnea and dysphagia. Laryngoscopy showed a mass occupying supraglottic, glottic and subglottic levels of the larynx. Cervico-thoracic computed tomography scan showed a laryngeal wall thickening with cervical lymphadenopathy. Laryngeal biopsy was performed. Microscopic analysis and immunohistochemistry confirmed the diagnosis of laryngeal lymphoepithelial carcinoma. Immunostaining for LMP1 was negative. Laryngeal lymphoepithelial carcinoma is an extremely rare and an aggressive tumor. It is rarely associated with the EBV. It must be regarded as a distinct entity. Radiotherapy is advisable as the unique therapy for local tumor. A correct diagnosis and a close collaboration between the pathologist and clinicians is mandatory for an optimal treatment strategy.

Unilateral pure trigeminal motor nerve neuropathy: A rare case report

Directory of Open Access Journals (Sweden)

Nishant K Srivastava

2014-01-01

Full Text Available Unilateral pure trigeminal motor nerve neuropathy is an extremely rare and unique condition, characterized by atrophy of the muscles, innervated by the motor branch of the trigeminal nerve. We report such a case in a 25-year-old male patient. The diagnosis was made on the basis of clinical and radiological examinations. Magnetic Resonance Imaging (MRI proved to be the key for establishing the diagnosis, which showed atrophy and fatty infiltration over the affected side of the muscles of mastication. We were unable to establish the cause of the condition even after performing a brain MRI.

Encephalocele presenting as lower lid swelling: A rare case report

Directory of Open Access Journals (Sweden)

Vaibhav Kumar Jain

2018-01-01

Full Text Available Encephalocele is a rare congenital abnormality characterized by abnormal protrusion of brain and meninges through an opening in the skull. We report an 8-year-old girl who presented with a swelling in the right lower lid for the last 6 years. In her infancy, she had undergone surgery for a very small swelling located in the right nasolacrimal area. On further clinicoradiological evaluation, anterior encephalocele was diagnosed. This case highlights the uncommon site of anterior encephalocele; misdiagnosis and mismanagement of which could result in dreaded complications such as meningitis and cerebrospinal fluid leaking fistula formation.

Ossifying fibroma in the sinonasal tract: a rare case report.

Science.gov (United States)

Charavarty, Shekhar; Datta, Debashish; Boro, Esha; Dutta, Bijita

2014-08-01

Ossifying fibroma involving the sinonasal tract is an extremely rare occurrence. We are reporting a case of ossifying fibroma in the left maxillary sinus in a 19-year-old female who presented with a mass in the left cheek and nasal obstruction. On examination, the swelling in the left cheek seemed to originate from the left maxilla and was smooth and bony hard. Patient underwent detailed radiological and image guided cytopathological investigations. Depending on the results of these tests complete surgical excision of the mass was done. Histopathological evaluation of the excised mass revealed it to be an Ossifying Fibroma.

Evisceration of Appendix through the Drain Site: A Rare Case Report.

Science.gov (United States)

Ravishankaran, Praveen; Rajamani, A

2013-06-01

Placing a drain after surgery is a usual procedure in any emergency abdominal operation. The drain is removed as soon as its purpose of draining the intraabdominal collection in served. Evisceration of intraabdominal organs through the drain site is a rare occurance. This case report is about an 12 year old girl who was admitted with blunt trauma abdomen. After completion of emergency laparotomy a drain was placed in the right lower quadrant. When the drain was removed on the 6th post operative day, the appendix eviscerated out of the drain site. The wound was extended a little and an appendectomy was done. This case is presented for its rarity as only two similar instances have been reported in literature so far.

Bilateral macular hemorrhage due to megaloblastic anemia: A rare case report

Directory of Open Access Journals (Sweden)

Sree Kumar Vaggu

2016-01-01

Full Text Available We report a case of a 17-year-old female patient who presented with sudden, painless, nonprogressive diminished vision in both eyes (best corrected visual acuity in right eye - 6/60 and left eye - 6/36. An ophthalmological evaluation revealed bilateral pale tarsal conjunctiva and bilateral macular hemorrhage. Hematological evaluation revealed the presence of megalocytic anemia (with hemoglobin - 4.9 g%. General examination showed severe pallor. On systemic examination, no abnormality was detected, confirmed by ultrasonography abdomen. Other causes of severe anemia have been ruled out. Intraocular pressure in both eyes was 12 mmHg. This case documents the rare occurrence of bilateral subinternal limiting membrane macular hemorrhage with megaloblastic anemia without thrombocytopenia and other retinal features of anemic retinopathy.

A rare case of splenic littoral cell angioma in a child

Directory of Open Access Journals (Sweden)

Recep Bedir

2014-01-01

Full Text Available Littoral cell angioma (LCA is a rare, benign primary vascular neoplasm of the spleen. The tumor originates from the littoral cells lining the sinuses of the red pulp of the spleen. Preoperative distinction of this tumor from other benign or malign splenic lesions is difficult. Radiologically most cases present as multiple nodules. Definitive diagnosis can only be made histopathologically and immunohistochemically following splenectomy. This clinical situation can coexist with various malignancies and autoimmune disorders. Even though, it is mostly benign, since it has the potential to become malignant after splenectomy, long-term follow-up is required. We present an LCA case, which appeared as a solitary mass in the spleen of an 11-year-old girl with abdominal pain admitted to our hospital.

High Origin of Radial Arteries: A Report of Two Rare Cases

Directory of Open Access Journals (Sweden)

Dong Zhan

2010-01-01

Full Text Available Variations in the arterial supply of the upper limb are relatively common, with reported prevalence rates ranging from 11 to 24.4%. Of these, the most commonly encountered variation in the arm is a high origin of the radial artery. However, after consecutively dissecting and examining 600 Singaporean Chinese cadavers (1,200 upper limbs, we found only two cases of this. In both cases, the brachioradial artery originated from the upper one-third of the brachial artery and continued distally as the radial artery in the forearm. The local prevalence of 0.33% of this variation is significantly lower compared against populations from other geographical regions. Although rare, recognition of the variation is of fundamental importance to clinical practice.

Mindful Self-Hypnosis for Self-Care: An Integrative Model and Illustrative Case Example.

Science.gov (United States)

Elkins, Gary R; Roberts, R Lynae; Simicich, Lauren

2018-07-01

The combination of mindfulness and self-hypnosis could provide a tool that is easily implemented by individuals who want to care for their well-being in times of high stress. Each discipline has been shown to be effective in relieving stress, and integration could further facilitate change while creating a tool that is highly accessible. There are many similarities between the two practices, such as focusing of attention and the emphasis on mind-body connection. However, important distinctions in psychological (e.g., self-monitoring) and neural (e.g., functional connectivity) elements are noted. A theory of how integrated mindful self-hypnosis may create change is presented. An illustrative case example of mindful self-hypnosis practice and a self-hypnosis transcript are provided.

A rare case of traumatic subretinal migration of crystalline lens, corroborated histologically.

Science.gov (United States)

Bawankar, Pritam; Das, Dipankar; Agarwal, Balmukund; Bhattacharjee, Kasturi; Tayab, Shahinur; Deka, Panna; Singh, Anshul; Borah, Erani; Dhar, Shriya

2017-12-01

Blunt trauma is the most common cause of posterior dislocation of the crystalline lens. We describe a rare case of subretinal migration of crystalline lens through the giant retinal tear following blunt ocular trauma. This incidental finding of subretinal dislocation of lens following blunt ocular trauma was confirmed on histopathological examination of the enucleated eye. This complication has not been described by histopathological examination in literature so far.

A rare case of traumatic subretinal migration of crystalline lens, corroborated histologically

Directory of Open Access Journals (Sweden)

Pritam Bawankar

2017-01-01

Full Text Available Blunt trauma is the most common cause of posterior dislocation of the crystalline lens. We describe a rare case of subretinal migration of crystalline lens through the giant retinal tear following blunt ocular trauma. This incidental finding of subretinal dislocation of lens following blunt ocular trauma was confirmed on histopathological examination of the enucleated eye. This complication has not been described by histopathological examination in literature so far.

A rare case of langerhans cell histiocytosis of the gastrointestinal tract

Institute of Scientific and Technical Information of China (English)

Uday Shankar; Monika Prasad; Om P Chaurasia

2012-01-01

Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an acute,fulminant,disseminated disease called LettererSiwe disease to solitary or few,indolent and chronic lesions of the bone or other organs called eosinophilic granuloma.Involvement of the gastrointestinal tract is very rare in LCH.We present the case of a 53-year-old woman referred by her primary care physician for a screening colonoscopy.A single sessile polyp,measuring 4 mm in size,was found in the rectum.Histopathological examination revealed that the lesion was relatively well circumscribed and comprised mainly a mixture of polygonal cells with moderate-to-abundant pink slightly granular cytoplasm.The nuclei within these cells had frequent grooves and were occasionally folded.Immunohistochemical staining was positive for CD-1a which confirmed the diagnosis of LCH.On further workup,there was no evidence of involvement of any other organ.On follow up colonoscopy one year later,there was no evidence of disease recurrence.Review of the published literature revealed that LCH presenting as solitary colonic polyp is rare.However,with the increasing rates of screening colonoscopy,more colonic polyps may be identified as LCH on histopathology.This underscores the importance of recognizing this rare condition and ensuring proper follow-up to rule out systemic disease.

Therapy-related assessment of self-harming behaviors in eating disordered patients: a case illustration.

Science.gov (United States)

Claes, Laurence; Vandereycken, Walter; Vertommen, Hans

2002-01-01

Understanding an individual's self-harming behaviors should be the basis for selecting person-specific therapeutic interventions. For that purpose, the assessment is aimed at identifying the self-harming behaviors and related symptoms as well as analyzing the external (situational) and internal (cognitive and emotional) conditions that contribute directly to the instigation of the self-harming behaviors. In this article, we demonstrate the use of a new assessment procedure that may guide the selection of therapeutic interventions. Data collection and processing are illustrated by an individual case study of an eating-disordered patient showing different types of self-harming behavior such as vomiting, alcohol abuse, cutting, and suicide attempts.

Total Pancreatic Fracture Due to Blunt Trauma: Report of a Rare Case

Directory of Open Access Journals (Sweden)

Kamil Gulpinar

2016-05-01

Full Text Available A rare case of pancreatic fracture due to blunt trauma was presented. The patient was 70 year old male who had a motor vehicle collision and was suspected a pancreatic trauma due his examinations with ultrasound and computerized tomography. The diagnosis of splenic injury and pancreas body total fracture in the point where the portal vein crosses the pancreatic body was made with the help of magnetic resonance cholangiopancreatography. He was taken to emergency surgery where a splenectomy and a distal pancreatectomy were performed. We represented this infrequent case of pancreatic fracture and its complications after blunt abdominal trauma and discuss the diagnostic and management practices.

Prenatal sonographic diagnosis of limb-body wall complex: case series of a rare congenital anomaly

Directory of Open Access Journals (Sweden)

Arshad Bhat, MBBS, DMRD

2016-06-01

Full Text Available Three case reports of a rare congenital anomaly “limb-body wall complex” also known as “body stalk syndrome” are presented with prenatal ultrasonographic diagnostic features, immediate after delivery evaluation, and histopathologic analysis.

The rare presentations of a large polyp and an esophageal carcinoma in heterotropic gastric mucosa: a case series

Directory of Open Access Journals (Sweden)

Alagozlu Hakan

2007-11-01

Full Text Available Abstract Background Heterotopic gastric mucosa (HGM is commonly seen in the upper esophagus during endoscopyand is generally considered a benign disease. A hyperplastic polyp and an adenocarcinoma arising in heterotopic gastric mucosa are quite rare occurences. Case presentations We present two cases: The first is a patient who suffered from dysphagia because of a large hyperplastic polyp that arose from HGM; the polyp was excised endoscopically. Secondly, we report a rare case of adenocarcinoma arising in HGM of the cervical esophagus. Conclusion Morphologic changes or malignant transformation can develop in the inlet patch. Therefore, gastroenterologists should be aware of the possibility of HGM just distal to the upper esophageal sphincter.

Distribution of rare-earths in solid solution crandalita- goyazita of Sapucaia (Bonito-Para)

International Nuclear Information System (INIS)

Costa, M.L. da; Melo Costa, W.A. de

1987-01-01

The Crandallite are predominant in the lateritic phosphates of Sapucaia, in the form of the solid solution Crandallite (Cn)- Goyazite (Gz)-Florencite (Fl). The Crandallite-Goyazite is predominant, where the maximum proportion of Florencite is Cn 60 Cz 34.8 Fl 5.2 - This proportion of Florencite is relatively high for laterites, and for this case having up to 1,374% weight of TR 2 O 3 in the total sample. The light rare elements are predominant over the heavy ores, and are illustrated in the distribution curve normalized for the chondrites. This curve is partially comparable with the curve for Apatite presents slight negative anomaly for the element Europium, and slight positive anomaly for The elements Thulium. The geochemical caracteristics for the rare earths in this group allow the prediction for the original rock for the laterites. (author) [pt

A Rare Renal Epithelial Tumor: Mucinous Cystadenocarcinoma Case Report and Review of the Literature

Directory of Open Access Journals (Sweden)

Abdulkadir Tepeler

2011-01-01

Full Text Available Primary renal mucinous cystadenocarcinoma is a very rare lesion of kidney which originates from the metaplasia of the renal pelvic uroepithelium. Only one case with primary mucinous cystadenocarcinoma has been reported in the English literature. We report second case of mucinous cystadenocarcinoma which was radiologically classified as type-IIF Bosniak cyst in peripheral localization. We aimed to present this extreme and unusual entity with its radiological, surgical, and pathologic aspects under the light of literature.

As a Rare Site of Invasive Fungal Infection, Chronic Granulomatous Aspergillus Synovitis: A Case Report

Directory of Open Access Journals (Sweden)

Aylin Canbolat Ayhan

2013-06-01

Full Text Available Aspergillus can causes invasive disease of various organs especially in patients with weakened immune systems. Aspergillus synovitis and arthritis are uncommon types of involvement due to this infection. Approches to fungal osteoarticular infections are based on only case reports. This paper presents a rare case of chronic granulomatous Aspergillus synovitis in an immunocompromised 5-year old girl who was treated for acute lymphoblastic leukemia.

Gelofen Induced Hypersensitivity: A Rare Case Report

Directory of Open Access Journals (Sweden)

M. Nikkhah Rankohie

2016-07-01

Full Text Available Introduction: Non-steroidal anti-inflammatory drugs (NSAIDs are drugs commonly pre-scribed in dental practice for the management of pain and swelling. But, rarely hypersensitiv-ity reactions are reported. Case Report: A 28 year old woman underwent periodontal plastic surgery (gingival graft. Postoperative analgesics (400 mg Gelofen ,oral and antibiotics were administrated for the patient. Three hours after discharge of patient, she complained of redness, itching , rapid swelling of her eyes in 10 minutes, and watery eye discharge 1 hour after taking the drugs. She was treated with 8mg/2ml mg Dexamethasone IM at the dental department and with Hy-drocortisone 100mg/ml IM and antihistamine drugs at the hospital. Conclusion: There are no published protocols and sensitivity and specifity of skin pick testing and patch testing for Gelofen. So avoidance of re-exposure is the best management strategy. The use of Cox-2 specific medications would be a proper alternative for pain relief. (Sci J Hamadan Univ Med Sci 2016; 23 (2:179-183

Urethral triplication with meatuses terminating on the penis: a rare case presenting with bifid urinary stream

Energy Technology Data Exchange (ETDEWEB)

Hirselj, Daniel A.; Jayanthi, Venkata R. [Nationwide Children' s Hospital, Department of Pediatric Urology, Columbus, OH (United States); Lowe, Gregory K. [The Ohio State University Medical Center, Department of Urology, Columbus, OH (United States)

2009-11-15

Urethral duplication is an uncommon clinical finding, and classification systems have been described to explain the clinical findings. Urethral triplication is an extremely uncommon diagnosis, with few published case reports. We present the rare case of an infant noted to have a bifid urinary stream and found to have urethral triplication on voiding cystourethrography. (orig.)

Urethral triplication with meatuses terminating on the penis: a rare case presenting with bifid urinary stream

International Nuclear Information System (INIS)

Hirselj, Daniel A.; Jayanthi, Venkata R.; Lowe, Gregory K.

2009-01-01

Urethral duplication is an uncommon clinical finding, and classification systems have been described to explain the clinical findings. Urethral triplication is an extremely uncommon diagnosis, with few published case reports. We present the rare case of an infant noted to have a bifid urinary stream and found to have urethral triplication on voiding cystourethrography. (orig.)

Ruptured Hemorrhagic Cyst of Undescended Ovary Mimicking Mucocele: A Rare Pediatric Case

Directory of Open Access Journals (Sweden)

Tamer Sekmenli

2017-01-01

Full Text Available Undescended ovary is a rare entity and usually presentedas a case report. It is associated with urinary and uterine anomalies. Symptomatic patients are diagnosed during surgery. Most of the patients are asymptomatic and treatment is unnecessary. They are incidentally diagnosed during infertility evaluation and treatment such as ovarian hyperstimulation studies. A 15-year-old female patient presented with the diagnosis of renal cystic massas identifiedduring ultrasonography in another hospital. Abdominal computedtomography image was requested. A cystic lesion of about 48×34 mm with well-defined borders associated with the appendix and probably with mucocele of the appendix was reported in the lower right abdominal quadrant close to the cecum. During exploration, the right ovary was seen to be attached to the cecum and was higher in position as well as a right ovary originated cystic structure of 5×5 cm.Using needle aspiration,intraovarian hemorrhage was confirmed and partial cystectomy was performed. The present study reports on an undescended ovary that hadacute abdomen symptoms imitating mucocele. In girls referring to the hospital with abdominal pain, although quite rare, undescended ovaries are to be also considered. As the incidence of renal and uterine anomalies is higher in suchpatients, in symptomatic cases relevant organs are to be investigated carefully during surgical intervention.

Pyogenic granuloma associated with periodontal abscess and bone loss - A rare case report

Directory of Open Access Journals (Sweden)

Bhrugesh J Panseriya

2011-01-01

Full Text Available A diverse group of the pathologic process can produce the enlargement of soft tissues in the oral cavity and often present a diagnostic challenge. This soft tissue enlargement may represent a variation of the normal anatomic structure, inflammatory reaction, cyst, neoplasm, and developmental anomalies. A group of reactive hyperplasias, which develop in response to chronic recurring tissue injury that stimulates an excessive tissue repair response. The pyogenic granuloma (PG is a reactive enlargement that is an inflammatory response to local irritation such as calculus, a fractured tooth, rough dental restoration, and foreign materials or hormonal (pregnancy tumor and rarely associated with bone loss. This paper presents a rare case of PG associated with periodontal abscess and bone loss in a 30-year-old male.

Pyogenic granuloma associated with periodontal abscess and bone loss - A rare case report.

Science.gov (United States)

Panseriya, Bhrugesh J; Hungund, Shital

2011-07-01

A diverse group of the pathologic process can produce the enlargement of soft tissues in the oral cavity and often present a diagnostic challenge. This soft tissue enlargement may represent a variation of the normal anatomic structure, inflammatory reaction, cyst, neoplasm, and developmental anomalies. A group of reactive hyperplasias, which develop in response to chronic recurring tissue injury that stimulates an excessive tissue repair response. The pyogenic granuloma (PG) is a reactive enlargement that is an inflammatory response to local irritation such as calculus, a fractured tooth, rough dental restoration, and foreign materials or hormonal (pregnancy tumor) and rarely associated with bone loss. This paper presents a rare case of PG associated with periodontal abscess and bone loss in a 30-year-old male.

A rare case of ectrodactyly in a child in India

Directory of Open Access Journals (Sweden)

Mishil S Parikh

2014-01-01

Full Text Available Ectrodactyly, termed split-hand/split-foot malformation (SHFM, is a rare genetic condition characterized by defects of the central elements of the autopod (hand/foot. Clinical presentation is with the absence of one or more median rays or digits creating cone-shaped clefts of the hands and/or feet. The present case of severe bilateral SHFM was presented in an 8-year-old girl in India. This case of SHFM involves the complete absence of the central rays of the autopod in which each of the hands and feet is divided into two parts by a cone-shaped cleft tapering proximally, resembling a "lobster claw." SHFM is often associated with other limb anomalies, including monodactyly, syndactyly and aplasia, and/or hypoplasia of the phalanges, metacarpals, and metatarsals. Most cases are sporadic; however, familial forms do exist with predominantly autosomal dominant inheritance. This case is an example of the non-syndromic form of SHFM expressed with isolated involvement of the limbs, while the syndromic form is associated with anomalies such as intellectual disability, ectodermal and craniofacial findings, and hearing loss. Non-syndromic isolated ectrodactyly does not usually require surgical intervention. We recommended against surgical reconstruction due to lack of evidence of functional disability.

Nonsyndromic Bilateral Multiple Impacted Supernumerary Mandibular Third Molars: A Rare and Unusual Case Report

Directory of Open Access Journals (Sweden)

G. Siva Prasad Reddy

2013-01-01

Full Text Available A supernumerary tooth is that which is present additionally to the normal series and can be found in any region of the dental arch. An impacted tooth is defined as the one which is embedded in the alveolus, so that its eruption is prevented, or the tooth is locked in position by bone or the adjacent teeth. The occurrence of multiple supernumerary teeth in only one patient in the absence of an associated systemic condition or syndrome is considered as a rare phenomenon. The occurrence of supernumerary teeth in the lower molar region is rare. A prevalence of less than 2% of cases occurring in this region has been estimated. Their occurrence presents a clinical problem for orthodontists and oral surgeons. The cause, frequency, complications, and surgical operation of impacted teeth are always interesting subjects for study and research. An impacted tooth can result in caries, pulp disease, periapical and periodontal disease, temporomandibular joint disorder, infection of the fascial space, root resorption of the adjacent tooth, and even oral and maxillofacial tumours. The management of impacted wisdom teeth has changed over the past 20 years from removal of nonsymptomatic third molars to simple observation. The aim of this paper is to present a rare case of bilateral multiple impacted supernumerary mandibular third molars.

Prehepatocholedochal proper hepatic artery. Rare anatomical variant. Surgical considerations. Case report.

Science.gov (United States)

Ardeleanu, V; Chicoş, S; Tutunaru, D; Georgescu, C

2014-01-01

In classical anatomic variants, the proper hepatic artery (PHA)continues the common hepatic artery (CHA) after the gastroduodenal artery (GDA) detaches itself and divides into the right hepatic artery (RHA) and left hepatic artery (LHA), the proper hepatic artery being located to the left of the hepatocholedochal duct (HCD). This paper presents an abnormal positioning of the PHA placed before the HCD with an increased diameter of about 5-7 mm, which could be confused with the HCD. We present the case of a 57 year-old woman diagnosed with acute lithiasic cholecystitis, associated with hypersplenism and hypertension. The literature mentions manifold anatomical variants of arterial liver vascularization,including PHA. For this reason, this paper presents an overview of similar cases that can be found in medical literature. The aforementioned case is a rare topographic anatomy for the PHA that can easily pass for HCD especially during celioscopy, therefore it is crucial for this to be acknowledged by all surgeons. Celsius.

Macrodystrophia lipomatosa – MR imaging of a rare congenital anomaly: review of 3 cases

Directory of Open Access Journals (Sweden)

Pallavi Aga

2010-12-01

Full Text Available Macrodystrophia lipomatosa (MDL results from progressive overgrowth of all the mesenchymal elements, with a disproportionate increase in fibro-adipose tissue. This rare congenital abnormality occurs most frequently in the distribution of the median nerve in the upper, and medial plantar nerve in the lower, extremity. Excess of unencapsulated fibro-adipose tissue on magnetic resonance imaging (MRI is virtually diagnostic and can differentiate this entity from other diseases with similar presentations. Involvement of the ulnar aspect of the hand, though rare, has been described; however, the lateral aspect of the foot as seen in our second case has not been reported, to the best of our knowledge.

[Rare bone tumour of the hand: capillary haemangioma of the second finger: case report].

Science.gov (United States)

Chbani, L; Benmlih, A; Hammas, N; Znati, K; Marzouki, A; Chbani, B; Boutayeb, F; Amarti, A

2011-04-01

Primary vascular tumours of bone are rare. Haemangiomas occur as incidental findings in the skull or in the spine. A solitary haemangioma of the hand skeleton is rare and difficult in radiological and histological differential diagnosis. We report a case of a 23 year-old patient presenting with a capillary hemangioma of the left second finger of few months duration for which he underwent amputation. Postoperative evolution was good and showed no recurrence. We discuss this through observation and a review of the literature including the different clinical, radiological and pathological diagnostic problems associated with an unusual site for vascular proliferation. Copyright © 2011. Published by Elsevier SAS.

Case Reports That Illustrate the Efficacy of SGLT2 Inhibitors in the Type 1 Diabetic Patient

Directory of Open Access Journals (Sweden)

David S. H. Bell

2015-01-01

Full Text Available SGLT2 inhibitors are only approved for use in adults with type 2 diabetes. However, because SGLT2 inhibitors have a mechanism of action that does not require the presence of endogenous insulin, these drugs should also be efficacious in type 1 diabetes where endogenous insulin production is greatly reduced or absent. Herein, I present five cases which illustrate the benefits of utilizing an SGLT2 inhibitor with type 1 diabetes. In these cases the use of SGLT2 inhibitors resulted not only in better glycemic control in most patients but also in some patients’ less hypoglycemia, weight loss, and decreased doses of insulin. In type 1 diabetes Candida albicans vaginitis and balanitis may occur more frequently than in type 2 diabetes. These cases show that a large randomized clinical trial of SGLT2 inhibitors in type 1 diabetes needs to be performed.

[Rare cause for severe hypertriglyceridemia - case 9/2013].

Science.gov (United States)

Kahl, Sabine; Roden, Michael; Mörike, Klaus; Müssig, Karsten

2013-11-01

We report on a 48-year-old female patient with recently developed severe hypertriglyceridemia. Medical history was remarkable for breast cancer with breast-preserving surgery and chemoradiotherapy. The patient has been treated with 20 mg tamoxifen per day for three months. Laboratory results showed hypertriglyeridemia, hypercholesterolemia and lowered HDL-cholesterol. Findings were consistent with a drug-induced hypertriglyceridemia caused by anti-estrogenic therapy with tamoxifen. After consulting the patient's gynaecologist, we discontinued tamoxifen treatment. Thereupon, triglyceride levels fell consistently. There were no signs of pancreatitis, serum amylase and lipase were in the normal range. Patients with pre-diagnosed metabolic disorders, especially dyslipidemia and type 2 diabetes, should undergo regular controls of serum triglycerides during tamoxifen treatment. Also, one should keep in mind that a subacute, severe rise in serum triglyceride levels may be caused, in rare cases, by tamoxifen treatment. © Georg Thieme Verlag KG Stuttgart · New York.

Gall bladder carcinoma with ampullary carcinoma: A rare case of double malignancy

Directory of Open Access Journals (Sweden)

Praveer Rai

2013-01-01

Full Text Available Simultaneous double cancers in the biliary system are rare. Most are associated with pancreaticobiliary maljunction (PBM. However, it can occur in patients without PBM. Differentiation between these events is important since these two mechanistic origins imply different stages of disease, as well as different subsequent treatments and prognoses. Herein, we report a case of ampullary carcinoma associated with gall bladder carcinoma diagnosed nonoperatively and palliated with biliary metal stenting.

Hemosuccus pancreaticus as a rare cause of gastrointestinal bleeding: a report of two cases.

Directory of Open Access Journals (Sweden)

Ghodratollah Maddah

2015-05-01

Full Text Available Pancreatic diseases are known to be associated with complications such as pseudocyst and abscess. A pseudoaneurysm associated with pancreatitis may develop as well. The pseudoaneurysm may rupture into various parts of the gastrointestinal tract; the peritoneal cavity, or the retroperitoneum. We report two cases of Hemosuccus pancreaticus admitted to our center in the past five years. One case was associated with acute pancreatitis, and another case was associated with chronic pancreatitis. A pseudocyst was found in two cases. Both were successfully managed by emergency surgery. So, Hemosuccus pancreaticus is a rare cause of gastrointestinal bleeding with difficult diagnosis, and surgery is the effective treatment for the patient with severe bleeding. However in cases with no life-threatening bleeding angiography and embolization can be performed.

A rare case of primary urachal mucinous adenocarcinoma

Directory of Open Access Journals (Sweden)

Ahmad Naufal Md Alwi

2017-12-01

Full Text Available Background: Urachal cancers arising from the urachus or its remnants, is a very rare and aggressive type of cancer in which occurrence is only about 0.01% of all adult cancers. Till date, there is no standard chemotherapy regimes or randomized trials due to the rarity of the disease. In 2007, not more than 350 written case reports in English has been published worldwide. This case report aims to share our department’s modest experience in management of a primary urachal mucinous adenocarcinoma in the adjuvant setting. Case Description: We report a 54-year-old gentleman from Kudat, Sabah, who presented with an umbilical mass and lower abdominaldiscomfort. Investigations revealed a primary urachal mucinous adenocarcinoma in May 2016 and he had undergone en-bloc resection of urachal tumour up to the serosal layer of the dome of bladder. Post-operatively, he received 12 cycles of adjuvant chemotherapy in the form of FOLFOX-4 regime from June 2016 to December 2016. He was last reviewed in September 2017 and is currently well and asymptomatic. Latest CT scan did not show any tumour recurrence nor distant metastasis. Results: This gentleman has been disease free for 16 months with no clinical or radiological evidence of local recurrence or distant metastasis. Conclusion: There are only few picaresque responses treating urachal cancers with chemotherapy regimens used for bladder carcinomas. Taking into consideration the enteric type histology of urachal adenocarcinomas, thechemotherapy regimes used for gastrointestinal malignancies may possibly yield better response and outcome.

Rare acquired hemostatic disorders as a cause of prolonged bleeding – presentation of two case reports

Directory of Open Access Journals (Sweden)

Polona Novak

2011-10-01

Full Text Available BACKGROUNDPatient’s anamnesis is of primary importance in determining hemostatic disorders. Based on anamnestic data, a clinician may decide for further laboratory tests. We must consider an acquired bleeding disorder in a patient with unusual, unexpected and prolonged bleeding episodes. In this article we will describe two rare acquired hemostatic disordes.TWO CASE REPORTSOur first patient had prolonged bleeding after a pacemaker implantation. We diagnosed him with acquired von Willebrand syndrome. Further on, the patient required a planned surgical procedure. In our second case we describe a patient with unusual and excessive skin bruising and prolonged bleeding after teeth extractions. He was diagnosed with acquired hemophilia.CONCLUSIONIn the assessment of a patient with a potential acquired bleeding disorder we must first rule out the most common causes, such as iatrogenic ones. But, because of high morbidity and mortality rates, we must also be aware of some rare acquired bleeding disorders. In case of uncertainty, we should consult with a hematologist.

A prenatally diagnosed pentalogy of cantrell case with encephalocele: A rare variant

Directory of Open Access Journals (Sweden)

Melih Atahan Güven

2009-06-01

Full Text Available AIM: The aim of this study is to present a prenatally diagnosed and postnatally confirmed Pentalogy of Cantrell case also with neural tube defect. CASE: Characteristic features of Cantrell Pentalogy are omphalocele due to the defect of anterior diaphragm and lower sternum, absence of pericardium and cardiac anomaly. We are presenting here a case with encephalocele and omphalocele containing the heart with atrioventricular septal defect detected during prenatal ultrasonography. There is no consanguinity and history of drug usage or toxin exposure during pregnancy. As these malformations cause a very low chance of survival, pregnancy was terminated after an informed consent. Postmortem genetic evaluation of the fetus confirmed the prenatal findings. CONCLUSION: It is easy to diagnose omphalocele during pregnancy but if it associates with heart anomalies, Cantrell Pentalogy must be remembered. Encephalocele and other types of neural tube defects very rarely associate with this disorder and there were fewer than 20 cases reported in the literature.

Rare case of multiple meningiomas in nonneurofibromatosis patient at unusual locations

Directory of Open Access Journals (Sweden)

Setia Vikrant

2017-06-01

Full Text Available Multiple meningioma is a condition in which more than one intracranial lesion is seen in different location and these lesions may occur with or without signs of neurofibromatosis. Incidence of multiple meningioma range from 1 to 10% in different series. We report a case of multiple meningioma in a 33 years old female who had 14 intracranial lesions both supratentorially and infratentorially, and underwent surgery for large right lateral intraventricular meningioma. She had two meningiomas located in posterior fossa associated with supratentorial meningioma, which has been rarely reported.

Aspergillus Salpingitis: A Rare Case Report

African Journals Online (AJOL)

rare entity, the correct diagnosis is of great importance for the indication of proper therapy. KEY WORDS: America ... Salpingitis is the most common serious infection in women .... immune systems, especially those recipients of stem-cell and.

Cranial dural arteriovenous fistula as a rare cause of tinnitus – case report

International Nuclear Information System (INIS)

Kuśmierska, Małgorzata; Gać, Paweł; Nahorecki, Artur; Szuba, Andrzej; JaŸwiec, Przemysław

2013-01-01

Tinnitus, occurring at least once in a lifetime in about 10–20% of the population, is an important clinical problem with complex etiology. Rare causes of tinnitus include cranial dural arteriovenous fistulas (DAVFs), which are usually small lesions consisting of abnormal connections between branches of dural arteries and venous sinuses or veins. Authors present a case of a 44-year-old woman with persistent, unilateral, treatment-resistant pulsatile tinnitus caused by a small dural arteriovenous fistula revealed in computed tomography angiography. Computed tomography angiography is a useful diagnostic method that in some cases allows for establishing the cause of unilateral, pulsatile tinnitus

Cranial dural arteriovenous fistula as a rare cause of tinnitus – case report

Energy Technology Data Exchange (ETDEWEB)

Kuśmierska, Małgorzata; Gać, Paweł [Department of Medical Radiology and Imaging Diagnostics, 4th Military Clinical Hospital, Wrocław (Poland); Nahorecki, Artur [Department of Internal Diseases, 4th Military Clinical Hospital, Wrocław (Poland); Szuba, Andrzej [Department of Internal Diseases, 4th Military Clinical Hospital, Wrocław (Poland); Medical University, Wrocław (Poland); JaŸwiec, Przemysław [Department of Medical Radiology and Imaging Diagnostics, 4th Military Clinical Hospital, Wrocław (Poland)

2013-07-01

Tinnitus, occurring at least once in a lifetime in about 10–20% of the population, is an important clinical problem with complex etiology. Rare causes of tinnitus include cranial dural arteriovenous fistulas (DAVFs), which are usually small lesions consisting of abnormal connections between branches of dural arteries and venous sinuses or veins. Authors present a case of a 44-year-old woman with persistent, unilateral, treatment-resistant pulsatile tinnitus caused by a small dural arteriovenous fistula revealed in computed tomography angiography. Computed tomography angiography is a useful diagnostic method that in some cases allows for establishing the cause of unilateral, pulsatile tinnitus.

Presentation of idiopathic retroperitoneal fibrosis at a young age: A rare case report.

Science.gov (United States)

Minocha, Priyanka; Setia, Ankur

2016-11-01

Abdominal pain is a very common symptom in all age groups but retroperitoneal fibrosis is a rare differential diagnosis suspected in young patients presenting with nonspecific abdominal pain and symptoms of obstructive uropathy. Presented here is a case of a 16-year-old boy who presented with symptoms of persistent abdominal pain and a previous history of swelling in the left leg. A computed tomography (CT) scan suggested retroperitoneal fibrosis and an exploratory laparotomy and histopathological examination were performed for definitive diagnosis. This case report is intended to promote awareness of retroperitoneal fibrosis in young patients among health care providers.

Cervical Cancer as a silent killer: A rare case report with review of literature

Directory of Open Access Journals (Sweden)

Deeksha Pandey

2015-01-01

Full Text Available Advanced-stage cervical cancer almost always presents either with abnormal vaginal bleeding or with foul-smelling vaginal discharge. We present here a rare case, where a postmenopausal lady presented almost silently with stage IVA cervical cancer. Fortunately, timely referral, correct diagnosis, and multispecialty team work could save her life.

Organizational Learning in Rare Events

DEFF Research Database (Denmark)

Andersen, Kristina Vaarst; Tyler, Beverly; Beukel, Karin

When organizations encounter rare events they often find it challenging to extract learning from the experience. We analyze opportunities for organizational learning in one such rare event, namely Intellectual Property (IP) litigation, i.e., when organizations take disputes regarding their intell......When organizations encounter rare events they often find it challenging to extract learning from the experience. We analyze opportunities for organizational learning in one such rare event, namely Intellectual Property (IP) litigation, i.e., when organizations take disputes regarding...... the organization little discretion to utilize any learning from past litigation success. Thus, learning appears be to most beneficial in infringement cases. Based on statistical analysis of 10,211 litigation court cases in China, we find support for our hypotheses. Our findings suggest that organizations can learn...

Idiopathic pleuroparenchymal fibroelastosis, a rare entity of interstitial pneumonia: A case report

Energy Technology Data Exchange (ETDEWEB)

Park, Jong Chun; Chae, Eun Jin; Song, Joon Seon [University of Ulsan College of Medicine, Asan Medical Center, Seou (Korea, Republic of)

2014-04-15

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described, very rare type of fibrotic interstitial lung disease predominantly involving subpleural areas of both upper lungs. IPPFE has distinctive radiologic and pathologic features: progressive subpleural opacity with fibrotic changes, predominantly in upper lungs, and dense elastic component on histology. We experienced one case of surgically confirmed IPPFE, with progression of radiologic findings on the serial CT examinations. We herein report the characteristic radiologic features of IPPFE with pathologic and clinical manifestations.

A rare case of chondroblastioma of the proximal tibia

International Nuclear Information System (INIS)

Gecov, P.; Terziev, I.; Hristoskova, R.; Georgiev, G.; Kinov, P.; Tivchev, P.

2011-01-01

The authors report a rare case of chondroblastoma which was localised in the proximal tibial epiphysis in a 14-year-old boy. The lesion was evaluated with routine roentgenography and computer tomography. The imaging techniques revealed eccentric lesion, predominantly in the medial part of the bone, periosteal reaction in the metaphysic, calcification and sclerosis. After diagnostic evaluation including biopsy, the lesion was treated surgically with curettage and grafting. He was followed up for two years with routine radiographs and physical examination. Two years after surgery the patient had no recurrence, no pain and had nearly full range of motion of the knee. Roentgenographic characteristics of the disease as well as differential diagnostics are discussed. In conclusion, imaging techniques should be supplemented with an open biopsy for the final diagnosis.

A rare occurrence of a steroid cell tumor of the pelvic mesentery: a case report

OpenAIRE

Louis Robert; Murhekar Kanchan; Majhi Urmila

2011-01-01

Abstract Introduction Steroid cell tumors are microscopically characterized by abundant eosinophilic or vacuolated cytoplasm that is often positive for fat stains. These tumors could be of ovarian or ectopic adrenal origin. We present a rare case of a steroid cell tumor arising from the pelvic mesentery. Case presentation A 31-year-old Asian woman was undergoing treatment for infertility and virilizing symptoms. She underwent laparoscopy followed by laprotomy for a suspected ovarian cyst/pelv...

Infiltrating Ductal Carcinoma Co-Existing with Intraductal Papillary Carcinoma of Male Breast: A Rare Case Report.

Science.gov (United States)

Kumar, Mayank; Pottipati, Bhaswanth; Arakeri, Surekha U; Javalgi, Anita P

2017-06-01

Male breast carcinomas are rare tumours, accounting for less than 1% of all malignancies in men. Intracystic Papillary Carcinoma (IPC) in males is a very rare entity, representing 5-7.5% of all male breast carcinomas. It lacks the classical clinical, radiological and cytological features of malignancy and usually presents as a benign-appearing lump. We report a case of Infiltrating Ductal Carcinoma (IDC) co-existing with intracystic papillary carcinoma in a 53-year-old male who presented with lump in the right breast.

CASE REPORT:Chondrosarcoma of Rib on Fine Needle Aspiration Cytology – A Rare Site

Directory of Open Access Journals (Sweden)

Prakash M Patil

2012-07-01

Full Text Available Background: Fine needle aspiration cytology (FNAC is effective in the diagnosis of bone tumors when combined with careful radiologic and clinical evaluation. Chondrosarcomas often arise in the pelvis or bones of the trunk, but primary chest wall (rib chondrosarcomas are relatively rare. Case Reports: This is a case of a patient with a chondrosarcoma arising in the left lower rib who underwent resection. The patient was a 30-year-old man with a 10x8x6cm tumor in the anterior chest wall of the left side lower rib. On Fine Needle Aspiration Cytology (FNAC of the mass on the anterior chest wall, a diagnosis of a low grade chondrosarcoma was made. This was confirmed histopathologically as a dedifferentiated chondrosarcoma. Macroscopically on excision of gray to pink lobulated mass with adjacent soft tissue and bone with foci of hemorrhage and necrosis the mass measured (M 10x 8x 6 cm. Incidence of chondrosarcoma peaks in the 5th to 6th decade and most commonly involves the femur, humerus, pelvis, and scapula. It rarely involves rib.

Laugh syncope as a rare sub-type of the situational syncopes: a case report

Directory of Open Access Journals (Sweden)

Nishida Katsufumi

2008-06-01

Full Text Available Abstract Introduction Laughter is a good medicine; it enhances cardiovascular health and the immune system. What happens, however, if a person laughs too much or the laughter becomes out of control? Laughter-induced syncope is rare and likely goes unrecognized by many health care providers. It is thought to be another form of Valsalva-induced syncope. Case presentation We report the case of a 56-year-old, moderately obese (body mass index of 35 man with a past medical history of sleep apnea, hypertension and hyperlipidemia who suffered from syncope secondary to intense laughter. The patient also had a history of syncope in the distant past when he collapsed on the floor for several seconds. Treadmill stress testing after the incident revealed no arrhythmia or ischemic disease, although he complained of dizziness after the test and a sudden drop in blood pressure was noted. Conclusion Laughter-induced or gelastic syncope is extremely rare. It is thought to be a sub-type of the situational syncopes.

Oral ulcer as an exclusive sign of gastric cancer: report of a rare case

Directory of Open Access Journals (Sweden)

Rimassa Lorenza

2005-09-01

Full Text Available Abstract Background The oral cavity is a rare but occasional target for metastases, which may masquerade as various benign and inflammatory lesions, and sometimes also be asymptomatic. Oral metastatic lesions have been described in various cancers, particularly lung, breast and kidney carcinoma. Case presentation We here describe an uncommon case of a hard palate mucosa and gingival metastasis from gastric carcinoma that was originally diagnosed as a periodontal disease. Histopathological examination of a biopsy of the lesion revealed a signet-ring cell carcinoma, and a subsequent biopsy of an ulcerated stomach lesion showed a poorly differentiated gastric carcinoma. The patient underwent gastric resection but died of heart failure on the tenth postoperative day; a post-mortem examination revealed a residual bilateral ovarian infiltration by gastric carcinoma (Krukenberg's tumor. Conclusion An occult carcinoma of the stomach may rarely metastasise to the oral cavity even as a first and exclusive manifestation; it is important to bear this possibility in mind because such conditions may mimic a benign disease.

A Rare Case of Primary Breast Mucosa- Associated Lymphoid Tissue Lymphoma

Directory of Open Access Journals (Sweden)

Marić Daliborka

2016-12-01

Full Text Available Breast involvement by lymphoma is uncommon and poses challenges in diagnosis. Breast involvement by malignant lymphoma, whether primary or secondary, is a rare event. Primary breast lymphomas account for 0.38% - 0.7% of all non-Hodgkin lymphomas, 1.7%-2.2% of all extranodal non-Hodgkin lymphomas, and only 0.04% - 0.5% of all breast cancer cases. Most frequent primary breast lymphomas are diffuse large B cell lymphomas (53%. Breast mucosa-associated lymphoid tissue (MALT lymphomas account for a small fraction of all the MALT lymphomas (1% - 2%. Herein we report a case of a patient with primary breast MALT lymphoma and its presentation on different imaging modalities. Two years after the presentation and treatment with eight cycles of chemotherapy, the patient is alive and well, without evidence of residual disease or recurrence.

A rare case of renal vein thrombosis due to urinary obstruction.

Science.gov (United States)

Jana, Tanima; Orlander, Philip R; Molony, Donald A

2015-08-01

Renal vein thrombosis (RVT) is an uncommon condition in adults and may be caused by endothelial damage, stasis, or hypercoagulable states. RVT is commonly identified in patients with nephrotic syndrome or malignancy. We present the case of a 57-yearold man with no past medical history who presented with a 1-month history of abdominal pain, dysuria, and hematuria. Initial laboratory studies were consistent with acute kidney injury (AKI). Imaging revealed bladder distension, enlargement of the prostate, bilateral hydronephrosis, and left renal vein thrombosis extending into the inferior vena cava. His renal failure and presenting symptoms resolved with placement of a Foley catheter and ureteral stent. The patient was discharged on anticoagulation. Here, we report a rare case of RVT that appears to have occurred as a consequence of obstructive uropathy causing massive bladder distention resulting in compression of the renal vein.

Osteosarcoma of the Maxilla: A Rare Case with Unusual Clinical Presentation

Directory of Open Access Journals (Sweden)

Pouyan Amini Shakib

2013-08-01

Full Text Available Osteosarcoma (OS is a malignant mesenchymal tumor, which rarely occurs in the maxilla. Although variable histologic and radiographic features of OS have been reported previously, in the majority of the cases painful swelling of the jaw is mentioned as the first clinical presentation. Furthermore, early diagnosis and wide surgical resection of the tumor are the most important determinant factors of prognosis. Therefore, the unusual clinical presentations of OS should be considered meticulously to expedite the diagnosis process. We describe a case of OS of the maxilla with extremely unusual presentation in a 42-year-old female, that was initially designated as “epulis fissuratum”. Here, we highlight the importance of combining the clinical, radiographic and histopathologic examination to obtain a definitive diagnosis and also the significance of early effective surgical intervention in evaluation of pathologic lesions.

Radiological Findings in a Case of Multiple Focal Nodular Hyperplasia Associated with Portal Vein Atresia and Portopulmonary Hypertension

Energy Technology Data Exchange (ETDEWEB)

Lee, In Joon; Jeong, Sook Hyang; Choi, Jin Woo; Park, Hee Sun; Lee, Kyoung Ho; Kim, Hae Ryoung [Seoul National University Bundang Hospital, Seoul (Korea, Republic of)

2008-08-15

We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities

Sustainable prevention of resource conflicts. New risks from raw materials for the future? Case study and scenarios for China and rare earths (Report 3.4); Rohstoffkonflikte nachhaltig vermeiden. Risikoreiche Zukunftsrohstoffe? Fallstudie und Szenarien zu China und seltene Erden (Teilbericht 3.4)

Energy Technology Data Exchange (ETDEWEB)

Taenzler, Dennis; Westerkamp, Meike [Adelphi Research, Berlin (Germany); Supersberger, Nikolaus; Ritthoff, Michael; Bleischwitz, Raimund [Wuppertal Institut (Germany)

2011-04-15

''Rare earths are to China what oil is to the Middle East,'' stated Deng Xiaoping in 1992 (Wang 2007). China accounts for 97 percent of global rare earth production, and as such the world is more dependent on it than it is on oil from the Middle East. That situation is significant because rare earths, although usually used only in small amounts, are of great strategic relevance. They are not only key components of many military technologies, including guided missiles and radar; they are also to be found in many high-tech products which we use in our daily lives - primarily electronic devices such as computer hard disks, plasma screens and MP3 players. They also make alloys harder, and are used to grind precision lenses. Rare earths are of particular importance in the field of environmental technology however. They are key components of catalytic converters, wind turbines, energy-saving bulbs and electric motors - and new applications in the environmental field are emerging all the time. These technologies are increasing the importance of, and the demand for, rare earths. As rare earths become ever more important, however, the question also arises as to the risks and opportunities they bring for consumers and producers. Consequently, this report (3.4) investigates those risks and opportunities, with a particular focus on China. Its analysis follows on from reports 1 and 2, illustrating and expanding upon their results. Likewise, this empirical case study will feed into the proposed solutions and recommended action to be set out in reports 4 and 5. This report is divided into a case study and four scenarios. The case study serves as an analysis of the status quo. It sets out potential conflict risks and opportunities arising from the situation as it exists in 2010. The subsequent four scenarios depicted were devised in the course of a Scenario Workshop in conjunction with a group of experts. They make use of the case study to set forth a range of

Acute abdominal pain presenting as a rare appendiceal duplication: a case report

Directory of Open Access Journals (Sweden)

Mahmood Ali

2012-03-01

Full Text Available Abstract Introduction Appendiceal duplication is a rare anomaly that can manifest as right lower quadrant pain. There are several variations described for this condition. We recommend aggressive operative management should this anatomical variation present in the presence of acute appendicitis. Case presentation We report the case of a 15-year-old African American girl who presented to our hospital with right lower quadrant pain and was subsequently found to have appendiceal duplication. Conclusion There are two categorical systems that have described and stratified appendiceal duplication. Both classification systems have been outlined and referenced in this case report. A computed tomography scan has been included to provide a visual aid to help identify true vermiform appendiceal duplication. The presence of this anatomical abnormality is not a reason for surgical intervention; however, should this be found in the setting of acute appendicitis, aggressive resection of both appendices is mandatory.