An adolescent female with underlying bilateral pseudophakia and idiopathic intermediate uveitis had reduced visual acuity due to posterior capsule opacification (PCO). The rapid progression and different morphological formations of Elschnig pearls were not influenced by the uveitis and immunosuppressive treatment. No capsulotomy was performed, and the PCO regressed spontaneously over time.
Classic Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder with bilateral massive painless cervical lymphadenopathy. It is a mysterious disease and there is little knowledge of its pathogenesis, clinical features, radiological findings, laboratory investigations, effective treatments and prognosis. Some of its clinical presentations may overlap with those of Mycobacterium tuberculosis infection. Just like tuberculosis infection, RDD may involve many other organs, for example, skin, kidney, bone, brain and spine. The diagnosis can easily be overlooked, especially in communities hyperendemic to tuberculosis infection. We report our experience in diagnosing and managing a patient with spinal RDD with concurrent tuberculosis infection, who was treated empirically for cervical tuberculous lymphadenitis without a conclusive laboratory finding prior to her spinal condition. In view of her acute neurological deficit, emergency spinal decompression was performed. Her intraoperative spinal samples had shown classic histopathological features of RDD. We believe the lymphadenopathy was part of the clinical presentation of RDD. She showed favourable neurological recovery throughout the follow-up.
Systemic lupus erythematous (SLE) is an autoimmune disease commonly treated with steroid which leads to immunosuppression and increased susceptibility to infection. Chronic laryngitis with whitish lesion on the true vocal fold in SLE may be caused by opportunistic organisms, such as tuberculous, fungal and Staphylococcus aureus infections. Videolaryngostroboscopy may be helpful in leading to the diagnosis and optimum treatment of glottic S. aureus A woman in her 40s with SLE presented with progressively worsening hoarseness for 2 months, accompanied by sore throat and odynophagia. Videoendoscopy showed erythematous and oedematous bilateral vocal fold with whitish lesion seen at the edge of middle one-third while the videolaryngostroboscopic evaluation showed there was severe asymmetry of the bilateral vocal folds, with severely reduced amplitude during phonation where the vocal cords were not vibrating, aperiodic vibratory cycles and 'always open', incomplete closure of vocal cord pattern. Later, endolaryngeal microsurgery and biopsy of the lesion confirmed of glottic S. aureus Her symptoms and followed up videolaryngostroboscopy showed resolution to normal findings after 6 weeks of cloxacillin. S. aureus infection of the glottis is a differential diagnosis in a chronic laryngitis with leucoplakic lesion in an immunosuppressive patient. Videolaryngostroboscopy has an important role in diagnosis, evaluation and treatment decision.
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.
A young adult patient with 46XX congenital adrenal hyperplasia (CAH) presented with recurrent painful haematuria. CAH was diagnosed at birth following ambiguous genitalia. Hormonal treatment was started, female gender was assigned and feminising genitoplasty was planned, however the patient was lost to follow-up. Gender dysphoria started to occur during childhood which prompted the family to raise the patient as a boy. He eventually identified himself as a male. Examination revealed a male phenotype with severely virilised genitalia. Imaging studies confirmed the presence of uterus with low confluent urogenital sinus. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed, and the troublesome symptoms were cured.
Congenital obstructive uropathy is a rare cause of ascites in infants. Majority of reported cases of genitourinary causes of ascites were due to posterior urethral valve. Here, we report a 6-month-old boy who presented with progressive tense ascites and peritonitis attributed by unilateral left distal ureteric obstruction and acute pyonephrosis. He underwent left nephrostomy placement, after which there was a remarkable improvement of ascites. He then underwent left ureteral diversion procedure a month later with a tentative plan for ureteral reanastomosis in 6 months. To date, there are no reports describing ascites secondary to distal ureteric obstruction beyond the neonatal period. The objective of this case report is to highlight unilateral urinary tract obstruction as a potential cause of transudative ascites. Additionally, the superimposed infection in the obstructed collecting system can lead to acute peritonitis likely due to translocation of bacteria into the peritoneal cavity.
Pheochromocytomas are rare in children. The diagnosis is usually established from a raised urinary or plasma catecholamine or their metabolites. We present a girl aged 11 years who manifested with a hypertensive crisis secondary to an adrenal tumour but with unexpectedly normal urinary metanephrine and catecholamine results. She improved spontaneously following the crisis and underwent surgery later. The histopathological study confirmed a pheochromocytoma with large central necrosis. Her genetic screening reported a pathogenic von Hippel-Lindau gene mutation. Surveillance scan postsurgery detected no other tumours. Following the catecholamine crisis, an acute infarct occurred, resulting in extensive tumour necrosis and subsequent rapid remission of symptoms and paradoxically normal biochemical markers. Although not unheard of in adults, we believe this is the first reported case of an extensive spontaneous necrosis resulting in a biochemically normal pheochromocytoma in a child.
We report an elderly man who presented with giddiness and right-sided weakness, constipation and constitutional symptoms for 6 months duration. Blood investigations indicated hypercalcaemia with normal serum phosphate and acute kidney injury. Serum intact parathyroid hormone was suppressed. CT revealed bilateral tiny lung nodules with right upper lobe tree in bud appearance and incidental findings of bilateral adrenal lesion. Tuberculosis was ruled out. CT adrenal showed multiseptated hypodense rim enhancement adrenal lesion bilaterally. Adrenal function tests were normal except for low dehydroepiandrosterone (DHEA). Right-sided cervical lymph node biopsy confirmed fungal infection with the presence of intracellular and extracellular fungal yeast. Serum cryptococcus antigen titre was positive. Our final diagnosis was disseminated cryptococcosis with lungs, bilateral adrenal gland and lymph nodes involvement. The patient was then treated with antifungal treatment. Serum calcium was normalised after 1 month with marked clinical improvement.
Primary amyloidosis is a rare systemic disorder often associated with multiple organ dysfunction. The most common form, light chain amyloidosis, has an estimated age-adjusted incidence of 5.1-12.8 cases per million person-years. Spine involvement is extremely uncommon. We present the case of a young Asian man with newly diagnosed amyloidosis involving the lumbar spine among multiple organs with a pathological vertebral fracture that required urgent spine surgery. We believe this is the first reported case to discuss the perianaesthetic challenges in the management of lumbar spine amyloidosis.
Lithium is a medication with a variety of medical usage for various diseases including bipolar mood disorder. As the therapeutic window of lithium is narrow, its usage is commonly associated with toxicity. Lithium toxicity affects multiple systems especially the central nervous system, leading to neuropsychiatric complications. Haemodialysis is an effective method for lithium removal especially in severe lithium toxicity such as neurotoxicity with electroencephalogram changes. We describe a case of lithium neurotoxicity with electroencephalographic abnormalities which was reversed following haemodialysis.
Osteoglophonic dwarfism, also known as osteoglophonic dysplasia (OD), is an uncommon skeletal dysplasia with an autosomal dominant mode of inheritance, which equally affects boys and girls. OD is saliently featured by craniosynostosis, dysmorphic facial features, impacted mandibular teeth, rhizomelic limb shortening and non-ossifying fibromas habitually at the metaphyseal regions, which usually disappear after skeletal maturity. The long bones in OD are portrayed by this distinguishable 'hollowed-out' appearance with metaphyseal cystic defects that have a natural history of spontaneous resolution. We report a case of a rare and unusual presentation of OD in a 23-year-old woman whom has been diagnosed with OD during her early childhood. She presented with a progressively enlarging right thigh swelling associated with pain for the past 1 year. Her right femur plain radiograph revealed diffuse lysis of the whole femur with cortical thinning. MRI revealed multiple bilateral femur benign cystic lesion synonymous with a severe spectrum of OD. She was started on a trial of oral bisphosphonates, which led to a significant improvement in pain.
Acute COVID-19 usually lasts 4 weeks from the onset of symptoms. We report two cases of COVID-19-associated organising pneumonia (OP) occurring beyond 4 weeks from the acute onset of symptoms. Both tested positive for SARS reverse transcription-PCR 2 months before presentation with a resolution of respiratory symptoms. The first case presented with residual fatigue and worsening exertional dyspnoea. Chest CT revealed an OP pattern. The second case presented with worsening cough and new-onset pleuritic chest pain with persistent radiological consolidation. A transbronchial lung biopsy confirmed OP. Both patients responded well to 12 weeks of steroid therapy. This case illustrates the rare presentation of OP as a late sequela of COVID-19 and the good response to steroid therapy.
Rituximab (RTX) is a monoclonal anti-CD20 antibody used to treat non-Hodgkin's lymphoma. RTX-organising pneumonia (RTX-OP) is a rare complication following treatment with RTX. We report a 49-year-old woman, with CD5-negative B-cell lymphoproliferative disorder who developed high-grade fever, dyspnoea and dry cough 3 days after the first dose of RTX. She responded poorly to antibiotics and antifungal therapy. High-resolution CT (HRCT) of the chest revealed bilateral patchy ground-glass opacities with arcade-like signs suggestive of OP. She was pulsed with intravenous methylprednisolone and RTX was discontinued. She was able to be weaned off the non-invasive ventilation (NIV) support and was discharged with maintenance prednisolone 1 mg/kg and tapered over 6 weeks. A repeated HRCT of the chest at 6 weeks showed a total resolution of OP. This highlights the early occurrence at day 3 of RTX-OP following the first dose of RTX and the complete resolution with steroid therapy.
A 33-year-old woman was diagnosed with right recurrent laryngeal nerve (RLN) schwannoma. She presented with a long history of hoarseness, and only recently developed dysphagia. On physical examination, a mass was observed over the right cervical level IV. Endoscopic examination of the larynx showed that she had right unilateral vocal cord palsy. She successfully underwent transcervical resection of the tumour followed by injection laryngoplasty. This study discusses the presentation of the tumour, radiological findings, our working diagnosis and treatment options of RLN schwannoma.
A woman in her 30s with underlying Graves' disease, who recently completed radioactive iodine treatment, presented with 2 weeks of acutely altered behaviour associated with auditory hallucinations and religious preoccupations. Laboratory investigation demonstrated elevated free thyroxine levels and suppressed thyroid-stimulating hormone levels. Additionally, there was a presence of antithyroid peroxidase antibodies consistent with autoimmune thyroid disease. She responded to antipsychotics and achieved biochemical euthyroidism. Subsequently, antipsychotic was tapered off during outpatient follow-up at the patient's own request, with supplement thyroxine continuing. After 1 week, acute hallucinations and religious preoccupations re-emerged, driving her to inflict self-injuries by swallowing coins and nails and banging her head against the wall, sustaining laceration wounds. Furthermore, she hammered a roofing nail into the external genitalia, embedded in the symphysis pubis. After supplemental thyroxine was stopped and olanzapine was started, she achieved biochemical euthyroid followed by remission of psychosis within 1 week. This case illustrates the importance of elucidating organic causes of psychosis as they are easily and swiftly reversible.