Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis preferentially targeting medium-sized arteries and not related with glomerulonephritis or small vessel involvement. Clinical manifestations of PAN are multisystem. The gastrointestinal, renal, cardiac, musculoskeletal, skin and central nervous systems may be involved. The aetiology remains unknown, and the ensuing vasculitis may lead to aneurysm formation and thrombosis in any organs of the body with resultant ischaemia. PAN of the intestines is a relatively common manifestation of this disease but rarely causes bowel ischaemia resulting in necrosis. Here we report a case of a young Chinese patient who presented with an acute abdomen requiring surgery and made good recovery post operatively. He remains free of symptoms while on steroid therapy.
Kodamaea ohmeri keratitis is an opportunistic pathogen seen in patients who have undergone invasive procedures and immunocompromised state. It has been identified in septicemia patients, resulting in mortality. To the best of our knowledge, we identified the first case of K. ohmeri keratitis following an injury with vegetative material. A 57-year-old woman with underlying, poorly controlled diabetes mellitus was gardening when a tree leaf accidentally poked her in the eye. Two weeks later, the patient presented with right eye pain, redness and progressive blurring of vision due to a traumatised right cornea. Slit-lamp examination showed a small inferior paracentral corneal stromal infiltrate with overlying epithelial defect. A corneal scraping sample yielded K. ohmeri from Analytical Profile Index (API) 20C yeast identification system. She was treated with intensive topical amphotericin B and fluconazole. After 6 weeks of treatment, the keratitis resolved with faint scar tissue, and her visual acuity improved.
Rosai-Dorfman disease (RDD) is a rare and benign lymphoproliferative disorder that commonly presents as painless, bilateral neck swelling. Extranodal presentations are considered rare, but the most common extranodal locations involved include skin, subcutaneous followed by nasal/paranasal sinuses. Although it is a benign condition, it may be mistaken as a malignant lesion and requires a biopsy for diagnostic confirmation. In this study, we report a rare case of RDD with bilateral neck node and nasal/paranasal sinus involvement which initially presented with bilateral nasal obstruction. And, we reviewed the management in this unusual case and discussed the helpful role imaging studies play in the further workup and subsequent follow-up to treatment response.
A rare syndrome of rapid-onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation (ROHHAD) has been recently described. We report the first patient with this syndrome in Southeast Asia and review reported cases to date. Our patient was good health with normal development until the age of 2. He then developed hyperphagic obesity, hypersomnolence, seizures, alveolar hypoventilation, central hypothyroidism, sodium and water dysregulation, gastrointestinal dysmotility, strabismus, disordered temperature and irregular heart rate, altered sweating, delayed puberty, mental retardation and recurrent respiratory tract infections. The cardiomyopathy with heart failure and abnormal cerebral spinal fluid (CSF) neurotransmitter analysis present in our patient have not been reported previously. Tumours of the sympathetic nervous system are known to be associated with this syndrome but had not been found in our patient at the time of reporting. We highlight the difficulty of achieving the diagnosis of ROHHAD syndrome and its overlap with other well-established disease entities. The mortality and morbidity resulting from the high incidence of cardiorespiratory arrest may be prevented by early ventilatory support.
We report a case of a 46-year-old Malay woman with underlying hypothyroidism post thyroidectomy who presented with worsening breathlessness, orthopnoea, productive cough and left-sided pleuritic chest pain of 3 days duration. Chest X-ray on admission showed left-sided massive hydropneumothorax. Pleural tapping revealed empyema. A chest tube was inserted immediately. The culture of pleural fluid grew Streptococcus constellatus The patient was treated with antibiotics for a total of 6 weeks and underwent open thoracotomy and decortication during admission. Subsequently, her lung condition improved and there was no evidence of recurrence during follow-up.
A 72-year-old Chinese man presented with mild symptoms of heart failure. Transthoracic echocardiography showed signs of cardiac tamponade though clinically he was relatively well. The option of pericardiocentesis was not carried out due to a narrow window for aspiration with only a thin layer of effusion seen surrounding the apex and right ventricle on subcostal view.Pericardial window was done via a left anterolateral thoracotomy. Intraoperatively, 500 cm3 of purulent fluid was drained. Microbiology screens were all negative. We present the atypical clinical course of this elderly man presenting with a large pyopericardium.
A previously healthy man presented with fever for 2 days and rapidly progressive purpuric rash for 1 day. He progressed into hypotension, disseminated intravascular coagulation and refractory shock despite resuscitation and early antibiotic commencement. Blood culture grew Streptococcus pneumoniae This case report highlights the fact that purpura fulminans can be a rare presentation of S. pneumoniae infection as well.
We report a case of a 19-year-old immunocompetent Malay woman who presented with a worsening psychotic disorder of 1-year duration. She initially presented with social isolation with subsequent mutism and stupor. Physical examination revealed a stuporous, emaciated, dehydrated woman with Glasgow Coma Scale of 11/15 (E4V2M5). She had a blank stare, mutism and akinesia. Motor examination revealed upper motor neuron findings. Neck stiffness was present, however, Kernig's and Brudzinski's signs were negative. There were no other findings on other systems. Brain imaging and EEG were normal. Cerebrospinal fluid investigations revealed positive cerebrospinal fluid Mycobacterium tuberculosis PCR (MTB PCR). The patient was treated with empirical antituberculosis drugs and steroids. On follow-up visit 1 month later, her psychotic symptoms had fully resolved. She was able to ambulate and care for herself; she was unable to recall the symptoms she had experienced before and during admission.
Complications of stroke can include neuropsychiatric symptoms. However, post-stroke psychosis is rare. We report a case where an acute presentation of psychosis, depression and fluctuating cognitive impairment in a middle-aged man turned out to be related to a silent brain infarction. The patient had a background of poorly controlled type 2 diabetes mellitus with glycated haemoglobin level of 9.0-11.0%, hypertension and ischaemic heart disease. His CT brain results showed multifocal infarct with hypodensities at bilateral lentiform nucleus and bilateral corona radiata. His strong genetic predisposition of psychosis and a history of brief psychotic disorder with complete remission 3 years prior to the current presentation might possibly contribute to his post-stroke atypical neuropsychiatric presentation, and posed diagnostic challenges. He showed marked improvement with risperidone 6 mg nocte, chlorpromazine 50 mg nocte and fluvoxamine of 200 mg nocte. The need of comprehensive treatments to modify his stroke risk factors was addressed.
A 57-year-old male chronic smoker with underlying diabetes mellitus presented with dysphonia associated with cough, dysphagia and reduced effort tolerance of 3 months' duration. Videoendoscope finding revealed bilateral polypoidal and erythematous true and false vocal fold with small glottic airway. The patient was initially treated as having tuberculous laryngitis and started on antituberculous drug. However, no improvement was observed. CT of the neck showed erosion of thyroid cartilage, which points to laryngeal carcinoma as a differential diagnosis. However, the erosion was more diffuse and appeared systemic in origin. The diagnosis of laryngeal perichondritis was made when the histopathological examination revealed features of inflammation, and the tracheal aspirate isolated Pseudomonas aeruginosa The patient made a good recovery following treatment with oral ciprofloxacin.
Rubinstein-Taybi syndrome (RTS) is a rare autosomal dominant disorder that occurs due to a microdeletion of chromosome 16p13. The craniofacial abnormalities in these patients may pose a challenge for anaesthetist performing tracheal intubation. However, there are no known reported cases of drug interaction with non-depolarising muscle relaxant in patients with RTS. This young patient with RTS presented with an unexpected prolonged atracurium effect during the course of anaesthesia. After ruling out other possible causes, we have come to a conclusion that RTS itself could have played a role in the prolonged effect of atracurium. However, further studies will be needed to confirm this hypothesis. In the meantime, care should be used when using muscle relaxants in patients with RTS.
A 55-year-old immunocompetent woman was presented with features of obstructive jaundice and a clinicoradiological picture suggestive of a hilar cholangiocarcinoma (Klatskin tumour). However, caseating granulomatous lesion associated with miliary nodules were revealed intraoperatively. The lesion responded to standard antituberculous therapy. This unusual presentation highlights the considerable diagnostic challenge in such case.
Plasma cell leukaemia (PCL) is an aggressive haematological malignancy which is classified into primary (pPCL) and secondary PCL. A 39-year-old Indian man presented to the Department of Hematology with a 2-week history of fever and lethargy. Clinically, he was pale and febrile. Haemogram revealed bicytopenia with leucocytosis. The peripheral blood film portrayed rouleax formation with 45% of circulating plasma cells. Serum protein electrophoresis and immunofixation revealed IgG lambda paraproteinaemia of 48 g/L. Bone marrow aspirate, flow cytometry and trephine were consistent with IgG lambda pPCL. He was treated with six cycles of bortezomib, thalidomide and dexamethasone combination chemotherapy followed by high-dose melphalan conditioning and autologous stem cell transplant. Currently, he is in complete remission for the past 18 months and is on oral lenalidomide maintenance therapy. Prognosis is often dismal in pPCL with the median overall survival below 1 year if treatment is delayed.
A 79-year-old smoker with a background history of a treated glottic carcinoma and chronic obstructive pulmonary disease presented with progressive hoarseness, symptoms of aspiration and shortness of breath for 6 months. Examination revealed an ulcero-fungating mass over the posterior commissure of the larynx. A tracheostomy, direct laryngoscopy and biopsy of the mass was performed to secure his airway and to exclude recurrent glottic carcinoma. Reassuringly, a histopathological examination of the mass revealed numerous fungal yeast bodies. He was then treated with itraconazole for 4 weeks and was followed up as and outpatient with complete resolution and no recurrence of the disease.
Primary primitive neuroectodermal tumour (PNET) and extraskeletal Ewing's sarcoma belongs to the Ewing's family of tumours. Primary tumours arising from breast are very rare. There are only a few case reports published on primary extraskeletal Ewing's sarcoma and PNET arising from breast. We present an extremely rare case of an inoperable primary Ewing's sarcoma arising from left breast with contralateral breast, lymphatic and lung metastasis.
Children with Down syndrome have a higher risk of stroke. Similarly, intravenous immunoglobulin (IV Ig) is also known to cause a stroke. We reported a 3-year-old boy with Down syndrome who presented with severe pneumonia and received IV Ig. He developed right hemiparesis 60 hours after the infusion. Blood investigations, echocardiography and carotid Doppler did not suggest vasculitis, thrombophilia or extracranial dissection. Brain computerised tomography (CT) showed acute left frontal and parietal infarcts. Initial magnetic resonance angiography (MRA) of cerebral vessels showed short segment attenuations of both proximal middle cerebral arteries and reduction in the calibre of bilateral supraclinoid internal carotid arteries. The boy was treated with enoxaparin and aspirin. He only had partial recovery of the hemiparesis on follow-up. A repeat MRA 13 months later showed parenchymal collateral vessels suggestive of moyamoya disease. We recommend imaging the cerebral vessels in children with a high risk of moyamoya before giving IV Ig.
Infantile haemangioma represents a congenital vascular anomaly commonly observed in the head and neck region. Such an occurrence over the postcricoid region, however, is rather unusual. Herein, the authors report a case of a synchronous postcricoid haemangioma in a 7-week-old newborn diagnosed with severe laryngomalacia. In addition to the floppy redundant arytenoid mucosa, flexible laryngoscopy revealed a lobulated bluish mass at the postcricoid. The lesion was hyperintense on T1-weighted sequence and was enhanced with contrast, supporting the diagnosis of a haemangioma. She underwent surgical excision of the haemangioma with intralesional steroid injection. Surveillance at 6-month postoperation did not show disease recurrence.
Postauricular swelling is usually encountered in an emergency setting in otorhinolaryngology, resulting from complication of acute or chronic suppurative otitis media. Besides that, postauricular swelling may occur secondary to various other conditions including infectious disease, tumour, vascular malformation, granulomatous condition and even trauma. Children less than 2 years old are prone to fall and up to 10% sustain traumatic brain injury without any obvious signs of neurological deficit. We describe a rare case of a postauricular swelling in a toddler which turned out to be salmonella extradural abscess from an infected traumatic haematoma. The importance of high clinical suspicion especially in a child with a history of fall cannot be emphasised more because a missed brain abscess could lead to potentially life-threatening problems. We would like to highlight that meticulous history taking along with prompt assessment and intervention is prudent for a better prognosis and recovery.