Pertussis remains a significant threat to young infants, even in an age of widespread vaccination. In its most severe form, malignant pertussis (MP), the infection can trigger a life-threatening immune reaction, including a high white blood cell count that can place significant stress on a baby’s heart and lungs. This clinical challenge is especially relevant in areas such as the Gulf Cooperation Council, where young demographics and rapidly advancing healthcare systems struggle with outbreaks that are often under-reported. In this context, we detail the case of a young infant who developed MP. The child’s course was fraught with extreme leucocytosis, respiratory failure and additional bloodstream infections acquired during hospitalisation. Early recognition, aggressive supportive care and vigilant monitoring are crucial; in addition, timely maternal immunisation is the strongest defence for newborns. The successful use of hydroxyurea to manage extreme hyperleucocytosis without the need for exchange transfusion or leucapheresis is a key educational feature of this case.
A man in his late 40s presented with progressive chest pain, cough and dyspnoea. Imaging revealed left lung collapse with pleural involvement. Thoracoscopic pleural biopsy confirmed pulmonary adenocarcinoma by immunohistochemistry. Staging fluorodeoxyglucose positron emission tomography-CT demonstrated nodal disease and unexpectedly diffuse marrow uptake with widespread lytic lesions involving the axial and appendicular skeleton, raising concern for a haematological malignancy. Serum protein electrophoresis revealed no monoclonal band, arguing against plasma cell dyscrasia. CT of the brain demonstrated multiple lytic calvarial lesions without parenchymal involvement, an unusual pattern in lung adenocarcinoma. The patient was diagnosed with stage IV lung adenocarcinoma with extensive skeletal metastases. Owing to poor performance status and advanced disease, the patient was managed with the best supportive care and died 5 months after diagnosis. This case highlights that lung adenocarcinoma may rarely present with diffuse marrow involvement and highlights the importance of histopathological correlation when imaging mimics haematological disease.
A woman in her 20s presented with neck pain and was diagnosed with a primary giant cell tumour (GCT) involving the C5 vertebral body and encasing the left vertebral artery (VA). Denosumab therapy was initiated to reduce tumour vascularity and facilitate resection. After preoperative embolisation of the left VA, total en bloc resection of the tumour was performed. Postoperatively, the patient experienced transient left upper extremity weakness, which improved significantly over time. Follow-up imaging showed successful bony fusion and no recurrence. This case highlights the importance of multidisciplinary planning in managing cervical spine GCTs involving critical neurovascular structures. Combined denosumab therapy and VA embolisation can enable safe and complete tumour resection, potentially improving outcomes and reducing recurrence risk, especially in young patients.
This report describes a rare case of spontaneous intracochlear haemorrhage causing unilateral sudden sensorineural hearing loss and vertigo in a male patient in his early 70s, initially suspected of suffering a stroke. We cover the importance of timing for establishing the diagnosis using imaging modalities and considerations for auditory rehabilitation in persisting hearing loss.
A woman in her 30s presented with recurrent massive haemoptysis and chronic respiratory symptoms, including fever, cough and breathlessness. She had previously received empirical anti-tubercular therapy without microbiological confirmation or clinical improvement. CT angiography revealed right pulmonary artery atresia with extensive bronchial and non-bronchial systemic collaterals, a partially thrombosed pseudoaneurysm arising from a left lower lobe segmental branch with associated pulmonary infarction and bilateral bronchiectatic changes. She underwent embolisation of the pseudoaneurysm and selected systemic collaterals. Subsequently, she developed progressive hypoxaemic respiratory failure and succumbed to her illness. This case highlights delayed recognition of unilateral pulmonary artery atresia in adulthood and the challenge of controlling life-threatening haemoptysis while preserving pulmonary perfusion.
Rhabdomyolysis, a rare condition in the paediatric population, is characterised by the release of intracellular components due to muscle damage. Although various aetiologies have been described, severe hypernatraemia is rarely recognised as a cause. We present a paediatric case of rhabdomyolysis associated with severe hypernatraemia secondary to traumatic brain injury (TBI). A male in early adolescence with severe TBI following a motorcycle accident developed severe hypernatraemia (168 mEq/L) during management in the intensive care unit with hypertonic solution. Creatine kinase levels were elevated up to 30 197 U/L, with transient acute kidney injury. Other causes of rhabdomyolysis were excluded. The patient recovered with conservative management without requiring dialysis. Severe hypernatraemia may be a risk factor for rhabdomyolysis in patients with TBI, potentially mediated by osmotic alterations and cellular damage. This case highlights the importance of monitoring renal function and creatine kinase levels in patients with TBI and electrolyte disturbance. This report underscores the need to consider hypernatraemia as a potential cause of rhabdomyolysis in paediatric patients with TBI. Vigilance and timely management are crucial for the prevention of complications.
Displacement of an intact mandibular coronoid process lateral to the zygomaticomaxillary complex (ZMC) is a rare complication of ZMC fractures. We report a case of a woman in her early 30s with malar flattening and inability to achieve full mouth closure following a fall from an electric scooter. Her CT scan revealed a comminuted right ZMC fracture with the ipsilateral intact coronoid process displaced laterally to the ZMC. This rare injury pattern produced mechanical restriction of mandibular movement in both opening and closure. Urgent operative management of the ZMC fracture allowed for spontaneous coronoid reduction allowing passive occlusion intraoperatively and active full mouth closure once awake. At the 3-month follow-up, mandibular movement and malar symmetry had fully recovered. This case highlights the importance of considering coronoid displacement in patients with midfacial trauma and mechanical jaw restriction, the value of CT and the role of early surgical management in preventing long-term dysfunction.
Trichorhinophalangeal syndrome (TRPS) is a very rare genetic disorder comprised of three recognised types, all characterised by short, deformed fingers with cone-shaped epiphyses (CSE) visible on radiographs. TRPS type I is the most common subtype. We report the case of a girl in her mid-teens who presented with the characteristic clinical and radiological features of TRPS type I. The diagnosis was confirmed by whole-exome sequencing, which identified a novel heterozygous variant in the TRPS1 gene. This case highlights a structured diagnostic approach to CSE and may further expand the mutational spectrum of TRPS type I.
We report the case of a woman in her late 60s who presented to our hospital with fever and general fatigue. Investigation revealed bicytopenia, splenomegaly and central hypothyroidism. Brain MRI revealed enlargement of the anterior pituitary lobe. A random skin biopsy demonstrated clusters of atypical lymphocytes within small vessels, leading to a diagnosis of intravascular large B-cell lymphoma (IVLBCL). The patient achieved complete remission following multi-agent chemotherapy and autologous peripheral blood stem cell transplantation, with normalisation of her endocrine function. This case represents a rare instance of IVLBCL presenting with hypopituitarism as a clinical manifestation.
A woman in her 70s was admitted three times over 22 months with recurrent pericardial effusions. A multiplex antinuclear antibody (ANA) assay by chemiluminescent enzyme immunoassay, reported only as a combined index, was repeatedly negative. Reassessment with careful history revealed long-standing Raynaud’s phenomenon and episodic abdominal bloating, prompting further evaluation for systemic sclerosis (SSc). Indirect immunofluorescence (IIF) on HEp-2 (human epithelial type 2) cells showed ANA positivity (fine speckled 1:640 and nucleolar 1:320) and ELISA detected anti-RNA polymerase III antibodies, confirming SSc. Because of pre-existing renal impairment and anti-RNA polymerase III positivity, glucocorticoids were avoided; colchicine 0.5 mg/day was started to prevent recurrence. No further pericardial effusion occurred during follow-up. This case illustrates that relying solely on a combined-index multiplex ANA screen can delay diagnosis and that HEp-2 IIF and disease-specific antibody testing are essential when clinical suspicion persists.
Levamisole, a synthetic imidazothiazole derivative, remains a cornerstone in the management of frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome in resource-limited settings. While its efficacy as a steroid-sparing agent is well-established, its safety profile is complicated by rare but severe autoimmune phenomena. We present two distinct paediatric cases of levamisole-induced vasculitis from India. The first case involves a female child in middle childhood who developed multisystemic vasculitis (pulmonary, renal and cutaneous) following an accidental dose escalation. The second involves a female child, also in middle childhood, who developed a similar phenotype after prolonged, stable therapy. Both cases were marked by the presence of anti-neutrophil cytoplasmic antibody and resolved promptly on drug withdrawal. These cases underscore that levamisole-induced autoimmunity is not confined to specific ethnicities or illicit drug contaminants but is a direct iatrogenic risk that clinicians must monitor closely in the paediatric population.
Evans syndrome is a rare autoimmune disorder characterised by concurrent or sequential autoimmune haemolytic anaemia and immune thrombocytopenia, often posing significant diagnostic and therapeutic challenges due to its relapsing nature and variable response to treatment. We report a patient with severe Evans syndrome who developed status epilepticus in the setting of intracranial haemorrhage secondary to profound treatment-refractory thrombocytopenia, illustrating the potential for life-threatening neurological complications when cytopenias are uncontrolled. Despite limited improvement with first-line therapies, the patient experienced haematologic recovery and clinical stabilisation following initiation of avatrombopag, a thrombopoietin receptor agonist. This case highlights the importance of early recognition of treatment refractoriness and supports the emerging role of avatrombopag as a valuable therapeutic option for persistent thrombocytopenia in Evans syndrome.
Pneumothorax of any cause typically represents a contraindication to air travel. Pneumothorax ex vacuo is often encountered in clinical practice due to non-expandable lung, a common finding in malignant pleural effusion (MPE). The presence of intrapleural air may prevent people diagnosed with terminal illness from participating in air travel. We present the case of a man with pleural mesothelioma who developed a large left hydropneumothorax following initial drainage of MPE. An indwelling pleural catheter (IPC) was placed on subsequent presentation. As the patient wished to undertake a domestic flight with his family, we established a protocol which allowed management of emergent symptoms and the in-flight risk of intrapleural air expansion. This case demonstrates that in carefully selected patients with MPE, pneumothorax ex vacuo and a functioning IPC, air travel may be feasible. Beyond the physiological considerations, the patient was able to fulfil a final trip with his family.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common inherited enzymatic disorder that predisposes blood cells to oxidative injury and haemolysis. It is typically diagnosed during childhood and affects males more frequently. We describe a patient presenting with profound acute haemolytic anaemia requiring transfusion following ingestion of fava bean-containing snacks. The patient presented with fatigue, dyspnoea on exertion, jaundice and dark urine. Laboratory evaluation revealed severe anaemia, elevated lactate dehydrogenase, indirect hyperbilirubinaemia and low haptoglobin levels, consistent with haemolysis. Autoimmune and infectious causes were considered unlikely after diagnostic evaluation. Initial and repeat testing confirmed G6PD deficiency. This case highlights the importance of considering G6PD deficiency in patients with unexplained haemolytic anaemia regardless of age, sex or ethnic background and underscores the need for dietary and medication counselling to prevent recurrent haemolytic episodes.
X-linked erythropoietic protoporphyria (XLEPP) is a rare non-acute porphyria associated with severe photosensitivity and, in some patients progressive hepatobiliary disease. We describe the perioperative management of a middle-childhood-aged female with XLEPP, end-stage liver disease, portal hypertension-related thrombocytopenia and coagulopathy who required urgent dental extractions to facilitate transplant preparation. Key anaesthetic considerations included avoidance of phototoxic light exposure, safe drug selection, altered pharmacology in liver disease and bleeding risk. Surgery was performed under propofol-remifentanil total intravenous anaesthesia using the hospital's iLED 7 (Baxter Medical Systems GmbH + Co. KG, Saalfeld, Germany) light-emitting diode (LED) theatre lighting system, without additional optical filters or intraoperative light dosimetry. Additional precautions included avoidance of sunlight and fluorescent lighting, theatre-blind closure, occlusive coverings during transfer and recovery, coagulation optimisation, tranexamic acid and meticulous local haemostasis. The patient had no perioperative phototoxic injury, haemodynamic instability or red cell transfusion requirement and was discharged home the following day. This case highlights multidisciplinary planning in rare paediatric photosensitive liver disease.
We report a case of a middle-aged man with no known comorbidities who presented with a 1-month history of fever, dry cough, weight loss and progressively worsening breathlessness. Initial investigations revealed declining haemoglobin, white blood cell and platelet levels, while a chest CT showed diffuse ground-glass opacities and a small pneumothorax. The patient was diagnosed with Evans syndrome (the co-occurrence of warm autoimmune haemolytic anaemia and bicytopenia) after a positive direct Coombs test and other haematological markers. Despite starting steroids, his fever persisted, leading to a further diagnosis of secondary haemophagocytic lymphohistiocytosis (HLH) after fulfilling six of eight HLH-2004 diagnostic criteria. Ultimately, disseminated tuberculosis was identified as the underlying trigger via endobronchial ultrasound-guided transbronchial needle aspiration of mediastinal lymph nodes. The patient successfully recovered following a combined treatment of anti-tubercular therapy and steroids.
A young woman presented with abdominal pain and jaundice secondary to Epstein-Barr virus-induced hepatitis and splenomegaly. She collapsed and CT imaging showed a large haemoperitoneum secondary to atraumatic splenic rupture. Splenic artery embolisation was attempted without success, and subsequently she underwent an open splenectomy. A collaborative multidisciplinary approach and adequate hospital set-up allowed for an initial attempt at non-operative intervention with the aim of splenic preservation, with rapid conversion to open surgery when necessary. Total blood loss of 7.5 litres was replaced with allogeneic and autologous blood products. Cell salvage was a key adjunct for supporting resuscitation and blood conservation intraoperatively. This case emphasises the need for careful planning of backup definitive intervention when attempting non-operative management in such cases, with discussion based on current evidence. We also highlight relevant factors when considering the use of intraoperative cell salvage as part of atraumatic splenic rupture management.
Myasthenia crisis is a lethal complication of myasthenia gravis, and about 20% of patients with myasthenia gravis may present for the first time in crisis. We present a case in which underlying, undiagnosed myasthenia gravis presented in crisis as acute-onset dysphagia. We would like to discuss the role of acute exacerbation of chronic rhinosinusitis and the administration of high-dose oral corticosteroids in the precipitation of the myasthenia crisis in this patient.
Management of thromboangiitis obliterans remains challenging, as revascularisation is often difficult to achieve and pharmacological treatments provide limited benefit. Sympathetic modulation through stellate ganglion neurolysis has been proposed as an alternative strategy to improve peripheral perfusion. A man in his 60s with significant smoking history presented with progressive ischaemic changes of the left hand, including digital cyanosis, severe pain and allodynia. CT angiography revealed segmental stenosis of the ulnar and interosseous arteries with preserved proximal vessels, consistent with thromboangiitis obliterans. Following ultrasound-guided stellate ganglion neurolysis, rapid clinical improvement was observed, including marked pain reduction Visual Analogue Scale (6–7 to 0–1), increased skin temperature and restoration of digital perfusion. Ultrasound-guided stellate ganglion neurolysis may provide rapid symptomatic relief and improve perfusion in patients with refractory ischaemia due to thromboangiitis obliterans. This approach may serve as a valuable adjunctive strategy, particularly in cases where conventional treatment options are limited.
