Idiopathic retroperitoneal fibrosis and ankylosing spondylitis – A new case report with review of the literature
Retroperitoneal fibrosis (RPF) is an uncommon disease (annual incidence estimated at 1/20,000–500,000) characterized by the development of dense fibrous tissue in the retroperitoneal compartment . RPF can be idiopathic or secondary to drugs, infections, radiotherapy, malignant diseases, major abdominal surgery, trauma and retroperitoneal injuries [2,3]. RPF has also been reported in patients with systemic autoimmune diseases including rheumatic diseases [4-7]. Several cases of RPF in patients with ankylosing spondylitis have been described. Herein, we report a new case of retroperitoneal fibrosis in a patient with ankylosing spondylitis.
Intra-Abdominal Desmoplastic Small Round Cell Tumor: A Diagnostic Dilemma In Resource-Constrained Settings
Introduction. Desmoplastic small round-cell tumours (DSRCT) are primarily intra-abdominal soft tissue sarcomas that are extremely rare and aggressive with a rapidly deteriorating clinical course and poor outcome despite therapy. It belongs to the family of small round blue-cell tumours, symptoms of the disease are non-specific and metastasis is often present at diagnosis. Objective. This report presents a typical case of an intra-abdominal DSRCT while highlighting how challenging the diagnosis can be in resource-poor settings and best-practice in the management of its attendant complications. Case presentation. This is a case 10-year-old boy who presented with a two-month history of weight loss and a two-week history of colicky abdominal pain, abdominal distension and vomiting and loss of appetite. He was pale, had left mandibular lymphadenopathy with a distended abdomen and ascites. A palpable mass was occluding ~80% of his rectal lumen. CT scan showed intra-abdominal masses in the rectovesical pouch and hepatic flexure with abdominopelvic lymphadenopathy Patient was worked up for exploratory laparotomy, colostomy placement and tumor biopsy, with the subsequent histology and immunohistochemistry a diagnosis of DSRCT was made. The patient developed moderate anaemia, acute kidney injury, hypertension and anarsarca while on admission; these were successfully managed with blood transfusions, intravenous fluids and drugs subsequently. He had a re-exploration for fecal disimpaction and adhesiolysis due to persistent intestinal obstruction. Patient was discharged 15 days after admissionin a stable clinical state. Conclusion. Desmoplastic round-cell tumour (DSRCT) primarily occurr within the abdomen, it is capable of mimicking the symptomatology of other intra-abdominal malignancies and a high index of suspicion is requisite to make a diagnosis especially in resource-poor settings with swift implementation of treatment modalities necessary to halt disease progress.
Unilateral Vestibular schwannoma with Pituitary macroadenoma- Rare concurrent dual intracranial lesion
The presenting clinic features of the cerebellopontine angle tumours and their management are well known, however there is limited knowledge regarding coexistence of other primary brain tumours which can be incidentally detected. A 70-year-old otherwise normal gentleman was referred from routine hearing screening programme centre with more of right sided hearing loss and occasional right sided tinnitus. Pure tone audiogram revealed an asymmetric high frequency sensorineural hearing loss .Gadolinium contrast enhanced MRI showed a right distal mass filling the entire internal acoustic meatus with an intracanalicular dural extension indenting the middle cerebellar peduncle with no significant enhancement. Additionally, an incidental enhancing asymmetric macroadenoma was detected in the pituitary sella indenting the left cavernous sinus without an extension and displacing the pituitary stalk to the right and displacing the carotids superiorly exerting insignificant pressure on the optic nerves. He had Hyperprolactinemia but normal visual fields. Here we present this rare concurrent presentation of dual intracranial primary tumours which by itself clinically is very uncommon.
Diffuse-type tenosynovial giant cell tumour of the temporo-mandibular joint: an indication for alloplastic joint replacement
A 25-year old female patient was referred to our department with a mass at the level of the right temporomandibular joint (TMJ). The patient experienced symptoms of paroxysmal pain of the TMJ and weight loss. Magnetic resonance imaging (MRI) revealed a tumour in the right masticatory space extending into the right TMJ, with invasion of the temporal bone. Surgical resection of the mass and total TMJ replacement surgery with a custom-made pros-thesis were performed. Final histological diagnosis of the resected specimen showed a dif-fuse-type tenosynovial giant cell tumour (D-TGCT). The multidisciplinary team’s decision was to give adjuvant radiotherapy; however, the patient declined further treatment. At 6 months follow-up, the patient had an excellent recovery with no signs of tumour recurrence on MRI. This is the first described case of D-TGCT treated with total TMJ replacement sur-gery using a custom-made alloplastic device.
Bilateral Distal Humeral Fracture in a patient with Osteogenesis imperfecta During the Covid Pandemic
Introduction: Osteogenesis imperfecta (OI) is a genetic disorder which is characterised by bone deformity, low bone mass, connective tissue manifestations and brittle bones with history of multiple fractures. Although recurrent fractures are known in patient with OI, the occurrence of bilateral simultaneous fracture of humerus is quite rare. Presenting complaint and investigations: The patient had a fall in the outdoors during a storm during the height of covid pandemic and sustained injury to both arms. Radiographs revealed comminuted fracture of left distal humerus and transverse extraarticular supracondylar fracture of right humerus. Diagnoses, therapeutics interventions, and outcomes: Although, conservative measures were advised as per the guidelines for orthopaedic surgery during the covid pandemic for most of the fractures, the decision to operate was made considering the morbid obesity and hampered mobility due to bilateral above elbow plaster.The patient underwent staged open reduction and internal fixation of both distal humerus with anatomical locking plates in a span of 2 weeks. She recovered uneventfully with a good functional range of movements and resumed her routine activities. Conclusion: The treatment for complex and unusual fractures should be individualised and should take into consideration of patient’s general condition and safety.
Long Progression-free-survival Pancreatic Carcinosarcoma Under Gemcitabine-nabpaclitaxel First Line Chemotherapy
Carcinosarcoma of the pancreas is a rare entity with short case series reported in the literature. Diagnosis is established by immunohistochemical examination including both carcinomatous and sarcomatous components. Prognosis is usually limited to 6-9 months life expectancy. Standard chemotherapy regimen is not well defined. We present a case of pancreatic carcinosarcoma with long-time progression-free-survival under first line chemotherapy treatment with gemcitabine-nabpaclitaxel combination. Tumor histopathological and clinical characteristics are reviewed.
71-year-old male patient with hypertension, obesity and personal history of prostate adenocarcinoma with blast bone metastases. Admitted after fall and pathological right pertrochanteric fracture. Programmed surgery is performed by means of endomedullary nailing on the fractured right hip and prophylactic left hip. Pre-surgical nasopharyngeal exudate for coronavirus is positive, with no apparent clinical translation. Three hours after surgery, the patient begins with dyspnea, central thoracic pain of a pleuritic nature and a feeling of imminent death. Physical examination reveals poor general condition, hypotension, diaphoresis, tachypnea and desaturation, without neurological affectation. Treatment is initiated with intensive serotherapy, oxygen therapy and analgesia. Analysis reveals troponin and normal NT-proBNP with elevated LDH and D-dimer, as well as mild metabolic acidosis. The electrocardiogram and transthoracic echocardiogram do not show any alterations. Urgent pulmonary CT-angiography targets signs compatible with fat embolism (see figure 1). The hemodynamic and respiratory instability improve progressively, not requiring admission to ICU, appearing at twelve hours bilateral axillary petechiae. Pulmonary fat embolism syndrome is rare, and can appear after fractures and long-bone surgery . The classic clinical triad is hypoxemia, neurological alteration and petechiae[²], due to the presence of fatty emboli in the circulation and pulmonary parenchyma.
A world in the head: Brain abscess caused by Paradoxical embolism due to pulmonary arteriovenous fistula as a debut of hereditary hemorrhagic telangiectasia
66-year-old patient with a history of alcoholism and smoking, chronic iron deficiency anemia treated with oral iron who was admitted for streptococcal brain abscess, during the etiological study we found arteriovenous shunt on echocardiography and pulmonary arteriovenous fistula, undergoing endovascular closure of the same during stay. In the physical examination, perioral teleangiectasis was observed, when meeting the Curaçao criteria, a genetic study of hereditary hemorrhagic teleangiectasia was requested, which was positive.
Male Sexual Assault – A Case Report of Foreign Body in the Rectum from Sexual Assault by Female Partner
Most rectal foreign bodies (FB) are a result of insertion for sexual gratification. FB in the rectum as. result of sexual assault in males by the female partner is rare. A 32-year-ol man presented with a 6-day history of deep anal pain and lower abdominal pain, following an assault by a female partner while he was drunk. Physical examination and plain radiography confirmed a FB in the rectum. The FB was removed at exploratory laparotomy following failed attempt at trans rectal removal under spinal anaesthesia. The FB was confirmed to be a perfume canister. The patient has remained well at two months of follow up. Male sexual assault by female is not uncommon and can lead to disastrous effects. The female sexual assault on male is underreported and reported usually when there are complications. There is need to do more in-depth study to investigate the incidence of this.
Rubinstein-Taybi syndrome (RSTS) is a congenital syndrome most associated with mutations on chromosome 16p13.3 that can result in both benign and malignant neurologic and hematologic neoplasms of various primary origins. We present the case of a 39-year old female with RSTS who presented with severe abdominal and pelvic pain. Abdominal and pelvic imaging revealed multiple masses involving the uterus, liver and spleen concerning for malignancy. Biopsies from the endometrium and cervix confirmed this as a poorly differentiated, widely invasive squamous cell carcinoma. This represents the first case of primary squamous cell carcinoma of gynecologic origin in a patient with Rubinstein-Taybi syndrome. This case aims to raise awareness of the gynecological malignancy in patients with RSTS as well as serves as a reminder to clinicians to have a broad differential diagnosis in all patients which may help lead to early recognition of pathology.