Clin. Pract., Volume 1, Issue 4 (October 2011) – 59 articles

Teratoma is the most commonly encountered germ cell tumour among the most common ovarian tumours; however, teratomas of the omentum and mesentery are extremely rare. They are usually asymptomatic or can produce compressive symptoms. The imaging features are suggestive. The present report describes such a case of primary omental teratoma encountered in a young patient, which was managed by surgical resection. The histopathological examination confirmed the diagnosis of mature cystic teratoma. Germ cell tumors are congenital tumors containing derivatives of all the three germinal layers, frequently seen in gonads. But their occurrence in extragonadal sites, such as omental teratoma, is relatively rare. Full article

We report a case of an inclusion cyst, resulting from female genital mutilation (FGM), which enlarged to such a degree that it restricted the patient’s movement. This report aims to raise the awareness of the medical community to the dangers that arise from a common remote complication of FGM. Full article

Osteopetrosis is a rare inherited disorder of bone causing increased bone density. Legg- Calvé-Perthes disease (LCPD), by contrast, is a more common idiopathic condition leading to variable avascular necrosis of the immature femoral head. We present a case of a 5-year-old boy presenting with these co-morbidities. We have found only one previous reference suggesting these two conditions can coexist in the literature. We discuss the basic principles of management of this interesting case. Full article

Solitary fibrous tumor is an uncommon neoplasm affecting adults and typically located in the pleura and can also occur in a large number of other extra thoracic sites. We present the case of a solitary fibrous tumor (SFT) of the retroperitoneum and describe their histopathological and immunohistochemical features. The identification of SFT in the retroperitoneum is of importance because its clinico-pathological behaviour is still unclear. The pathologist plays a fundamental role in establishing both the positive and differential diagnosis. Full article

We report a case of congenital heterotopic salivary gland with draining sinus in the lower neck on the right side of a 10-year-old female, which we initially thought to be a branchial fistula. Heterotopic salivary glands are rare lesions in the neck and when present appear very similar to branchial cleft sinus or fistula. This congenital lesion is rare. This is probably the first report from India. It is important to report this case to raise the awareness of this condition. Full article

Adamantinoma is a rare primary bone tumor that commonly arises in the jaw and has also been described in the appendicular skeleton such as the tibia. We report 2 cases of tibial adamantinomas that were originally misdiagnosed; one as fibrous dysplasia of the tibia and the other as a cutaneous eccrine carcinoma in a groin mass, which was metastatic adamantinoma to the inguinal lymph nodes. Such metastatic adamantinoma to the groin lymph nodes is extremely rare. The clinical and pathological data with a review of the available literature on inguinal lymph node metastases from primary tibial adamantinoma are reported. Increased clinical awareness and accurate recognition of such uncommon patterns of inguinal nodal metastases are imperative for appropriate planning of therapeutic strategies and risk management in these patients. Full article

Duodenal lipomas are relatively uncommon and are rarely responsible for clinical symptoms. Occasionally, searching for aetiology of gastrointestinal bleeding leads to the final diagnosis of duodenal lipomas. Here, we present the case of a 68-year-old woman who suffered with repeated melena and weight loss. Endoscopy, abdominal computed tomography (CT) and histopathological outcomes are described in this case of duodenal lipoma with ectopic duodenal glands. Full article

Anomalous origin of coronary arteries is also a relatively rare congenital malformation and has been reported as the cause of angina pectoris and arrhythmia. Long QT syndrome (LQTS) is a rare inherited arrythmogenic disease characterized by susceptibility to lifethreatening arrhytmias andsudden cardiac death. We present a 36-year-old patient in whom two rare anomalies coexist and treated succesfully with β-blocker therapy. Full article

A 65-year-old man was admitted as for one month of repetitive dizziness and one episode of syncope. Electrocardiogram showed sinus bradycardia and his Holter monitoring also showed sinus bradycardia with sinus arrest, sino-atrial block and a longest pause of 4.3 s. Then sick sinus syndrome and Adam-Stokes syndrome were diagnosed. Then a dual chamber pacemaker (Medtronic SDR303) was implanted and the parameters were normal by detection. The patient was discharged 1 week later with suture removed. Then 1.5 month late the patient was presented to hospital once again for sudden onset of chest pain with exacerbation after taking deep breath. Pacemaker programming showed both pacing and sensing abnormality with threshold of?5.0V and resistance of 1200?. Lead perforation was revealed by chest X-ray and confirmed by echocardiogram. Considering the fact that there was high risk to remove ventricular lead, spiral tip of previous ventricular lead was withdrew followed by implantation of a new ventricular active lead to the septum. Previous ventricular lead was maintained. As we know that the complications of lead perforation in the clinic was rare. Here we discuss the clinical management and the possible reasons for cardiac perforation of active ventricular lead. Full article

The differential diagnosis of sudden hearing loss is very extensive. However, in most patients, no underlying pathology will be found and the patient is consequently diagnosed with idiopathic sudden hearing loss. Nevertheless, it is important that one stays alert in everyday routine practice to less common but potentially treatable pathology. We report a case of a 53- year old patient with sudden unilateral hearing loss and tinnitus caused by otosyphilis. Diagnosis, therapy and the incidence of (oto)syphilis are discussed. Also, as ENT-surgeons, it is important to realise that syphilis is still with us, and that its prevalence might well increase in the near future. Full article

The rate of re-rupture of Achilles tendon after surgical treatment were reported to 1.7-5.6% previously. Re-rupture of Achilles tendon generally occurs subcutaneously. We experienced two rare cases of the open re-ruptures of Achilles tendon with a transverse wound perpendicular to the primary surgical incision. Re-rupture occurred 4 and 13 weeks after surgical treatment. We suggest that open re-rupture correlates more closely with skin scaring and shortening. Another factor may be adhesion between the subcutaneous scar and the suture of the paratenon and Achilles tendon with post-operative immobilization. Full article

When the platelet count falls below 20¥ 109/L, the risk of spontaneous life threatening hemorrhage is concerning for both physician and patient. When medical management fails, splenectomy is often used to manage the severe thrombocytopenia before spontaneous, life-threatening gastrointestinal or intracranial bleeding occurs. We present the non surgical management of such a patient with refractory sever thrombocytopenia who refused surgical intervention. She underwent partial splenic embolic therapy with 500-700 ? particles. Her platelet count spontaneous recovered from less than 20¥109/L to normal range. Her counts remained in normal range after discharge home. Further study is needed to determine the most appropriate role for embolization in patients who are refractory to medical management and those with chronically low platelet counts requiring frequent plate transfusions for invasive procedures. Full article

Myxomas are mostly seen in the 2th and 3rd decades. They rarely occur in childhood and maxillofacial region is rarely involved. The recurrence incidence is high. We report this unusual case occurring in a 9-year-old girl in the maxillofacial region and recurrence four months after initial treatment. Full article

Extraosseous Ewings tumor (EES) is a rare entity. Few cases have been reported in literature. There are no specific guidelines for management of this disease. We are reporting a case of EES arising from left lateral abdominal wall. We did wide excision of tumor followed by chemoradiation. Patient is asymptomatic after 8 months of follow up. Full article

Upper gastrointestinal (GI) bleeding is defined as bleeding proximal to the ligament of Treitz. The most important aspect of management of GI bleeding is to locate the site and cause of bleeding. The aim of the study is to find out the common etiology, presentation and management, including the role of upper GI endoscopy. Recent advances have meant that endoscopic hemostatic methods are now associated with a reduced rate of re-bleeding, cost, blood transfusion, length of hospital stay and mortality. A prospective study of 50 cases was carried out between August 2001 and July 2003. Patients with signs and symptoms suggestive of upper GI bleeding (UGIB) such as hematemesis, melena, aspirated blood from nasogastric tubes, profuse hematochezia, etc., were included in the study. The patients were selected randomly. The most common cause of UGIB in the present study was acute erosive gastritis (34%) followed by portal hypertension (24%) and peptic ulcer (22%). All 50 patients underwent upper GI endoscopy, of whom 39 patients were treated conservatively and 11 patients underwent endotherapy to control bleeding. Out of 39 patients treated non-endoscopically, 6 cases required laparotomy to control UGIB. 8 of 50 cases had past history of UGIB, 5 of whom had a previous history of endotherapy. One case was treated with devascularization as routine hemostatic methods failed. So, initial method of choice to control the bleeding was endotherapy and surgery was undertaken if an endoscopic method failed. The most common cause of hematemesis in our setting was acute erosive gastritis followed by portal hypertension. Endoscopy is a valuable minimal invasive method to diagnose and treat upper GI bleeding. Full article

Retroperitoneal liposarcoma presenting as an inguinal hernia is a rare entity. We present the first case of Giant recurrent liposarcoma presenting as a recurrent inguinal hernia in a 40-year-old male. Physical examination showed an irreducible lump in the right inguinal region and a scar in the right lumbar and right inguinal region. Computed tomography (CT) scan of abdomen revealed it to be a retro peritoneal mass extending into the right inguinal region along and involving the cord structures. Wide local excision of the tumour with right orchidectomy and inguinal hernioplasty was performed. Histo-pathology confirmed it to be a liposarcoma. Patient received postoperative radio therapy. Follow up of two years has shown him to be disease free. Retroperitoneal liposarcoma can grow along cord structures into the inguinal canal and mimic an irreducible indirect inguinal hernia. Full article

X-linked adrenoleukodystrophy (X-ALD) is a rare inherited metabolic disorder, in which accumulation of very long chain fatty acids (VLCFAs) results in damage to the central nervous system. As the disease is X-linked, males are affected severely, but female carriers may also present with neurological symptoms. We report the case of a young adult female, who presented with episodic sensorimotor symptoms. Although she was a heterozygous female carrier of X-ALD, subsequent investigations confirmed a diagnosis of multiple sclerosis (MS). To the best of our knowledge, this is the first reported case of a female X-ALD carrier in which the clinical features were more consistent with co-existent MS than ALD-related pathology. The case serves as a reminder that alternative, more common diagnoses should also be considered in carriers of rare neurological syndromes. Full article

Syncope in a patient with a Brugada syndrome channelopathy carries significant prognostic implications and warrants consideration of implantable cardioverter defibrillator (ICD) implantation. We report a case of a 62- year-old gentleman who presented with a transient loss of consciousness and an electrocardiogram (ECG) suggestive of type 1 Brugada syndrome. Further investigation revealed evidence of a silent myocardial infarction and negative ventricular tachycardia stimulation and Ajmaline testing. Careful review of the ECG’s subsequently showed the type 1 pattern was present in only V1. Full article

Testicular leiomyosarcoma is a rare tumor. It may arise secondarily following exposure to radiotherapy, chronic inflammation, or usage of high dose anabolic steroids. However, in absence of risk factors, it is rarely seen. Only 15 cases of Primary Intra testicular leiomyosarcoma have been reported in world literature. We present a case of testicular tumor in an elderly male. Preoperative work up showed raised Lactate Dehydrogenase (LDH) levels. He underwent high orchidectomy. Histopathology and immunohistochemistry confirmed it to be a primary intra testicular leiomyosarcoma. A brief case report with review of literature is presented. Full article

Orbital apex syndrome is commonly been thought to have a poor prognosis. Many cases of this syndrome have been reported to be caused by paranasal sinus mycosis. We encountered a very rare case (60-year-old woman) of sinusitis with orbital apex syndrome due to Pseudomonas aeruginosa infection. She had received insulin and dialysis for diabtes and diabetic nephropathy, moreover anticoagulants after heart by-pass surgery. She underwent endoscopic sinus operation and was treated with antibiotics, but her loss of left vision did not improve. Recently, sinusitis cases due to Pseudomonas aeruginosa were reported to be a increasing. Therefore, we should consider the possibility of Pseudomonas aeruginosa as well as mycosis as infections of the sinus, especially inpatients who are immunocompromised body. Full article

Nephrogenic systemic fibrosis (NSF) is a recently identified idiopathic cutaneous fibrosing disorder that occurs in the setting of renal failure. The disease initially called nephrogenic fibrosing dermopathy is closely linked to exposure to gadolinium-based contrast media used during magnetic resonance imaging in patients with renal insufficiency. Although little is known about the pathogenesis of this disease, the increased expression of transforming growth factor-beta has been demonstrated recently. Herein, we present a case of NSF was partially treated due to a moderate and temporary response to plasmapheresis with no recurrence for 6 months, but returned at the end of 6^th month. Full article

Achondroplasia is the commonest form of short-limbed dwarfism and occurs in 1:26,000- 40,000 live births. This is an autosomal dominant disorder with abnormal endochondral ossification whereas periosteal and intramembranous ossification are normal. The basic abnormality is a disturbance of cartilage formation mainly at the epiphyseal growth plates and at the base of the skull. The anesthetic management of achondroplastic dwarfs is a challenge to the anesthesiologist. Both regional as well as general anesthesia have their individual risks and consequences. We report a case of an achondroplastic dwarf in whom combined spinal epidural anesthesia was used for fixation of a fractured femur. The patient had undergone previous femur surgery under general anesthesia since he had been informed that spinal anesthesia could be very problematic. There was no technical difficulty encountered during the procedure and an adequate level was achieved with low-dose local anesthetics without any problem. Postoperative pain relief was offered for three consecutive postoperative days using epidural tramadol. We discuss the anesthetic issues and highlight the role of combined spinal epidural anesthesia with low-dose local anesthetics in this patient. This approach also helped in early ambulation and postoperative pain relief. Full article

In immunocompetent hosts, symptomatic cytomegalovirus (CMV) infection occurs in around 10% and has traditionally been considered to have a benign, self-limiting course. Active CMV infection in immunocompetent persons is very rare. However, manifestations of CMV infection in immunocompromised hosts have been extensively reported.We present a case of a 58-year-old immunocompetent woman with a primary cytomegalovirus infection, which presented as a severe gastroenteritis and hepatitis. Full article

Actinomycosis has been recognized to be associated with the use of intrauterine contraceptive device. We are reporting a case where a patient with severe pelvic actinomycosis presented with the clinical picture of an ovarian tumour. A 44-year-old lady attended the A&E with progressively worsening lower abdominal pain. A computerized tomography (CT) scan showed the presence of a large pelvic mass, right hydronephrosis and prominent para-aortic lymph nodes and an elevated C-reactive proteine (CRP) and white cell count (WCC). When there was no improvement with antibiotic therapy, a laparotomy was performed, where bilateral tubo-ovarian abscess and dense adhesions were found. A subtotal hysterectomy, bilateral salpingoophorectomy and small bowel resection was performed. Histopathology of the specimen confirmed the diagnosis of actinomycosis. The case highlighted the diagnostic dilemma for ascertaining the nature of the pelvic mass in this patient. Due to its invasion of surrounding tissues and the formation of masses severe infection is often confused with an ovarian neoplasm. Full article

A 63-year-old woman presented with progressive renal insufficiency, until a glomerular filtration rate (GFR) of 12 mL/min. A renal biopsy demonstrated glomerular deposition of immunoglobulin k light chain. The presence of a small population of monoclonal plasmacytes producing an only light k monoclonal component was demonstrated and Bortezomib and Dexamethasone (BD) was provided as initial therapy. After seven courses of therapy, renal function improved without dialysis requirements up to a GFR 31 mL/min. Under hematological complete response (HCR) the patient underwent high dose of melphalan (HDM) and autologous peripheral blood stem cell transplant. Fifty-four months later the patient remains in HCR and the GFR has progressively improved up to 48 mL/min. This report describes a notably renal function improvement in a patient with Light Chain Deposition Disease after therapy with BD followed by HDM, which can support this treatment as a future option for these patients. Full article

A palmar dislocation of scaphoid and lunate is uncommon. We have found only 19 reported cases in the literature. We reported a simultaneous, divergent dislocation. The closed reduction followed by percutaneous pinning has given a good result without avascular necrosis of any carpal bone. Full article

Benign osteoblastoma (OB) is a rare tumor of bone representing less than 1% of all tumors of the maxillofacial region. Vertebral column, sacrum, long bones, and calvarium are more frequent sites of this tumor. Clinically, patients present with pain and swelling. Histologically, contain a well vascularized, osteoblastic connective tissue stroma and occasionally, osteoclasts along with osteoid and varying degrees of calcification, as well as immature bone may also be noted. The main aim of this report is present a case of OB occurring in the mandible and a brief review of English Medical Literature of this tumor. Many bone-producing lesions possess some overlapping clinical, radiographic or histopathological findings similar to osteoblastoma. Understanding and correlating all features helps in correct diagnosis and adequate management of this rare entity. Hence, osteoblastoma has to be differentiated from other bone lesions for correct diagnosis. Full article

Watershed infarcts (WI) evolve in hemodynamic risk zones. Clinical picture of WI can be associated to partial epileptic seizures. Diffusion weighted brain magnetic resonance imaging (MRI) allows a clear diagnosis. WI pathogenesis involves either embolic or hemodynamic mechanism. A 69-year old patient presented with sub-acute occurrence of right hemiparesis and partial epileptic seizures of the right arm. Carotid ultrasounds demonstrated occlusion of the right extra-cranial internal carotid artery (ICA) and tight stenosis of the contralateral ICA. Brain Diffusion-Weighted magnetic resonance revealed acute ischemic lesions within the watershed area of the left hemisphere. Our case supports the hypothesis of impaired washout of emboli in low-perfusion brain areas as the mechanism underlying cortical WI. Full article

Carcinosarcoma is a rare malignant ‘mixed’ tumour in the head and neck region. We present a case of carcinosarcoma in a long standing parotid lump and share our experience in the management of the disease together with a review of recent English literature on the subject. Full article