Background: PVNS is a rare, benign & aggressive disorder arising from either synovial joints or tendon sheaths; it may erode articular structures and bones. We present a case with unique features of PVNS being extra-synovial and by this report we open a gate for more researches in this field. Case Presentation: This case report concerns a 35-year-old female with a history of right knee pain for 6-month duration proceeded by gradual swelling over posterior aspect of the knee, she denies any history of trauma, clinical examination was unremarkable but apart from tenderness over the infrapatellar region with full flexion. MRI shows a heterogenous signal extra-articular and extra-synovial lesion in posterior aspect of the knee suggesting Pigmented Villonodular Synovitis, FNA revealed a hemosiderin-laden macrophages and multinucleated giant cells, Tru-cut biopsy result was suggesting PVNS as synovial cells were seen admixed with hemosiderin-laden macrophages with fibroblastic elements. Through posterior approach; the lesion was surgically excised and histopathological examination confirmed the diagnosis, the lesion was recurrent after 1-year and MRI revealed the same features, the lesion was excised by arthroscopic intervention. Conclusion: We concluded that PVNS cannot be excluded when extra-synovial lesion is assessed, and further researches on this topic will expand our understanding of the etiological and pathological aspects of this tumor.
Identification of Interictal Paroxysmal Diffuse Sharp Activity with Eye Closure in Patients with Generalized Epilepsy
Interictal EEG recordings of patients with generalized epilepsy have known interictal abnormalities such as generalized spike and wave activity during photic stimulation and hyperventilation, interictal spike and wave or diffuse sharp activity . We report three patients with confirmed generalized epilepsy who’s interictal recordings showed paroxysmal diffuse sharp 10 Hz activity in all leads with eye closure following eye blinking. This pattern was not associated with interictal generalized spike and wave activity, clinical change in the patient or did not follow seizure activity. Abnormal eye movement with generalized spike and wave activity has been described in Jeavon’s syndrome, eyelid myotonia and Sunflower syndrome. However, our patients did not meet criteria for any of these diagnoses. Therefore, we feel that our finding of paroxysmal diffuse sharp alpha activity is a novel finding in these patients with primary generalized epilepsy and may be a newly reported marker for patients with primary generalized epilepsy. Recognition of PDSA activity and further study of this pattern is encouraged.
Intestinal anastomosis blowout following post-operative cardiopulmonary resuscitation: A case report
Background: Pneumoperitoneum following cardiopulmonary resuscitation (CPR) is a very rare complication with a challenging management. In this paper we describe the management of a patient who suffered a blowout of his colorectal anastomosis after undergoing CPR for a cardiac arrest in the early post-operative period. Additionally, we present a thorough literature review of the management of CPR-related pneumoperitoneum. Summary: Five days after a sigmoid resection for colon cancer, a 71-year-old male went into pulseless electrical activity and CPR was initiated, with complete clinical recovery. After CPR the patient was found to have new hydropneumothorax and pneumoperitoneum. Because he had a normal abdominal examination, lack of leukocytosis, and no evidence of a bowel perforation on water-soluble CT imaging, the patient was initially managed non-operatively with close clinical follow-up. However, he failed the non-operative management and ultimately required a laparotomy demonstrating a blowout of his colonic anastomosis. Conclusion: Physicians should remain aware of the risk of damage to fresh bowel anastomoses following CPR. There should be a low threshold for surgical exploration in patients that develop CPR-related pneumoperitoneum soon after intestinal surgery, even when patient’s clinical status is stable.
Perioperative visual loss (POVL) is a drastic complication that can result after ocular and more commonly non-ocular surgery, mostly reported after spine and cardiac bypass procedures. Despite the rarity of such complication, it has been reported following the abdominal surgeries. Overall, the most common cause of POVL is ischemic optic neuropathy but any pathology to the optical system from the cornea to the occipital lobe can lead to this rare phenomenon. Here, we are reporting the second case in the literature of post-operative visual loss after laparoscopic appendectomy. A young female, with no underlying disease, underwent laparoscopic appendectomy after septic shock secondary to acute appendicitis. Postoperatively, patient complained of complete blindness after extubation. Neurologic examination revealed bilateral near complete blindness, and hemodynamic ischemic stroke in the occipital cortex.
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.