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Hyperreactio luteinalis is a benign, pregnancy-related condition with cystic enlargement of the ovaries and elevated androgen. However, only one-third of patients manifest as maternal virilisation and rarely does it cause fetal virilisation. Here, we report a virilised baby girl born to a virilised mother because of hyperreactio luteinalis. This case illustrates our management to maternal and fetal virilisation.

Pulmonary arteriovenous malformations (PAVMs) are rare vascular lesions characterised by abnormal connections between the pulmonary artery and vein bypassing the pulmonary capillary bed and causing right-to-left shunt. Paradoxical embolism is known to occur in these cases, leading to inoculation of septic focus in the systemic circulation. We report a case of multiple PAVMs who presented clinically with seizures and altered sensorium. On radiological work-up, multiple brain abscesses and large PAVMs were evident. The patient was successfully treated by endovascular embolisation of the PAVMs using a vascular plug and multiple coils. The patient showed complete clinical recovery and resolution of brain abscesses on follow-up.

In this paper, we report the case of a man in his 30s with a first-episode psychosis, characterised by a subtype of delusional misidentification syndrome in which the delusion of doubles is exclusively of the patient’s own self. This subdivision can be termed ‘syndrome of doubles of the self’ or ‘syndrome of subjective doubles’. Additionally, an examination of the patient’s mental state showed paranoid delusions. After being evaluated in the emergency department, the patient was hospitalised, and medicated with antipsychotic drugs. One week later, he was discharged with total remission of psychotic symptoms. This is a rare and curious presentation of a psychotic episode, with very few similar cases described to date.

Gorham-Stout disease (GSD) is thought to be due to uncontrolled proliferation of vascular and lymphatic structures within bone tissue causing destruction and osteolysis of bone. We present a patient in her mid-40s who reported chronic shoulder pain, a pleural effusion and irregular periods. Investigations showed osteolysis of her ribs, pleural effusions, an ovarian mass and a raised carbohydrate antigen 125 (Ca-125). She was subsequently diagnosed with GSD, and referred to gynaecology-oncology in consideration of potential ovarian malignancy. GSD is a diagnosis of exclusion that requires a high degree of clinical suspicion, as well as multiple investigations to achieve diagnosis. Clinicians rely on a small number of case reports to provide guidance for this. Therefore, this report provides an overview of a rare pathology, considers the differentials of a raised Ca-125 and describes how a pleural effusion, which links them both, alarmed us regarding an incidental finding of an ovarian cyst.

Autoimmune disorders are common presenting manifestations of immunodeficiency syndromes. We present a case of a woman in her late teens, with a history of frequent sinopulmonary tract infections during her childhood, who presented to our hospital with anaemia, jaundice and fatigue. She also had significant physical growth retardation for her age and sex. With this case report, we intend to present the diagnostic and therapeutic challenges faced by the patient and our healthcare system and propose a few feasible solutions to tackle these challenges.

Plurihormonal pituitary adenomas are rare forms of pituitary adenomas that express more than one hormone. The most common association is with growth hormone (GH) and prolactin. Cosecretion of GH and adrenocorticotrophic hormone (ACTH) is rare with only 25 reported cases in literature. Most presented with features of GH excess, and only four presented with Cushing’s disease. We report a case of a woman in her 30s with recurrent plurihormonal pituitary macroadenoma cosecreting GH and ACTH, diagnosed during workup for polycystic ovarian syndrome, and both times presenting uniquely with Cushing’s disease. Biochemical testing showed GH excess and hypercortisolism. She underwent transsphenoidal surgery twice and immunohistochemistry showed positive staining for GH and ACTH on both occasions. We aim to raise more awareness of this rare type of pituitary adenoma, shed light on the importance of recognising rare presentations and highlight the necessity of rigorous follow-up given morbidity and potentially higher mortality risk.

Werner syndrome (WS), also known as adult progeria, is a rare autosomal recessive inherited progeroid syndrome characterised by multiple features consistent with accelerated ageing. This disease is associated with several rheumatic conditions such as early osteoarthritis and osteoporosis, sarcopenia, soft-tissue calcifications, gout, limb ulcers and scleroderma-like skin features. WS should be included in the differential diagnosis of systemic sclerosis. The diagnosis is clinical, and in 90% of cases, a genetic test reveals a pathogenic variant of the WRN gene.WRN encodes a member of the RecQ family of DNA helicases and has a role in DNA repair. 86 different pathological WRN mutations have been identified so far. Here we present a case report of a typical WS patient associated with a newly described genetic variant of the WRN gene.

Patients with severe anaemia who refuse or cannot safely receive red cell transfusion present challenges during pregnancy, delivery and the postpartum period. Strategies including HBOC-201 (Hemopure) and intraoperative use of cell salvage have been used in non-pregnant patients to improve oxygen carrying capacity; however, these products pose unique risks in pregnant patients, those with sickle cell disease (SCD) and those undergoing caesarean section (C-section). We describe a case of a pregnant sickle beta+thalasasaemia patient who presented at 27 weeks gestation with pre-eclampsia and severe anaemia. As a Jehovah’s Witness, she declined allogenic blood transfusion. The patient successfully underwent emergent C-section with cell salvage and received HBOC-201 immediately after delivery, during the operative procedure. To our knowledge, this is the first published report documenting a Jehovah’s Witness patient with SCD who successfully received cell salvage and then HBOC-201 immediately postdelivery.

Two patients with an acute left-sided colonic obstruction had a successful decompression after construction of a blowhole transverse colostomy as a bridge to surgery. However, they presented with two rather unknown stoma-related complications during this bridging period. Patient A had a stomal prolapse with additional skin problems.Patient B complained of abdominal discomfort during follow-up. The blowhole colostomy appeared to be stenotic. Stoma dilation and irrigation was initiated to prevent complete closure.Definite resection of the left-sided obstruction and reversal of both blowhole colostomies was successful, and the patients recovered without further complications.We hypothesise that incision size may be related to prolapse and stenosis rates and that eversion of the mucosa of the blowhole may reduce the risk of stomal stenosis.

The increasing prevalence of severe obesity is a major public health concern. Bariatric surgery is an important treatment option for severe obesity due to its long-term sustained result. Multiple studies have shown that patients have an increased risk of developing inflammatory bowel disease following bariatric surgery. Takotsubo syndrome usually presents as acute left ventricular systolic dysfunction without corresponding obstructive coronary artery disease after an acute stress episode. We describe a unique case of a patient who developed de novo ulcerative colitis and takotsubo cardiomyopathy shortly after sleeve gastrectomy. The patient made a successful recovery due to prompt recognition and appropriate treatment.

A patient with a surgical history including an historic laparoscopic adjustable gastric band (LAGB) procedure presented to the emergency department with a history of haematemesis, intractable vomiting and abdominal pain 3 days following an outpatient oesophagogastroduodenoscopy (OGD) for chronic anaemia. A day after presentation to the emergency department, following admission, the patient underwent a repeat OGD. In contrast to her previous procedure, this demonstrated abnormal fluid and food deposition within what was originally reported to be a large hiatus hernia with a tight lower oesophageal sphincter. On corroboration with CT imaging obtained, in retrospect the endoscopic findings were attributed to slippage of the patient’s adjustable gastric band. The band was loosened via its subcutaneous access port, following consultation with the surgical service, resulting in complete resolution of the patients’ symptoms and she was discharged. This case is an example of gastroscopy as a rare trigger for the common complication of slipped gastric band following LAGB.

Autoimmune glial fibrillar acidic protein (GFAP) astrocytopathy typically presents as acute or subacute meningoencephalitis with or without myelitis. We describe a case of autoimmune GFAP astrocytopathy that mimicked tuberculous meningitis. A man in his 70s was referred to our hospital with lethargy persistent for 2 months, appetite loss for 1 month and fever with headache for 10 days. The cerebrospinal fluid test revealed lymphocytic pleocytosis with elevated adenosine deaminase (ADA). Laboratory investigations ruled out microbial and neoplastic causes. Empirical therapy for tuberculous meningitis combined with corticosteroid improved the patient’s condition. Culture forMycobacterium tuberculosisfailed to show microbial growth despite 1 month of incubation. The cerebrospinal fluid was examined for GFAP antibody and returned positive result. Antituberculous agents were discontinued, and corticosteroid was administered until patient’s symptoms resolved completely. Thus, clinicians should consider autoimmune GFAP astrocytopathy as one of the differential diagnoses of lymphocytic meningitis with elevated ADA.

Pulmonary mucosa-associated lymphoid tissue (pMALT) lymphomas are rare, representing 80%. Thus, an index of suspicion and early detection are vital. This case highlights that pMALT should be considered in patients with non-specific symptoms and coeliac disease. Bronchoscopy is a valuable diagnostic tool to be used in these cases.

Phakomatoses are a group of congenital disorders characterised by hamartomatous lesions of the skin as well as the central and peripheral nervous systems. The presence of naevus flammeus or port-wine stain is a characteristic feature of many such disorders including Sturge-Weber syndrome (SWS), Klippel-Trenaunay syndrome (KTS) and Phakomatosis pigmentovascularis (PPV).We describe the ocular findings in a patient with coexisting PPV, SWS and KTS

Pulmonary arteriovenous malformations (PAVMs) are rare and often asymptomatic vascular anomalies that can be associated with serious neurological consequences due to right-to-left shunting. We report a case of a woman in her 80s without substantial medical history who presented with a headache, weakness and personality changes, and was found to have a pyogenic brain abscess requiring emergent neurosurgical evacuation. The abscess grew oral flora, suspected to have reached the brain via an incidentally discovered PAVM. With drainage and antibiotics, the patient achieved a full recovery and the PAVM was embolised. To our knowledge, this is the oldest presentation of a PAVM-associated brain abscess in the published literature. Older patients may present without the typical signs and symptoms of a given illness, which complicates accurate diagnosis and treatment. Primary care physicians can help facilitate timely care and positive clinical outcomes.

A patient in her late 50s with antiphospholipid syndrome presented to general ophthalmology clinic for annual hydroxychloroquine retinopathy screening. She had taken 400 mg hydroxychloroquine daily for over a decade. She denied any visual changes and visual acuity was 20/20. Her examination and fundus photos were normal, but macular optical coherence tomography of the right eye demonstrated inner retinal atrophy and visual field tests revealed a corresponding paracentral scotoma, consistent with a prior cilioretinal artery occlusion. Prior testing from visits with other ophthalmologists revealed that this occlusion had occurred previously, but she had only been informed of not having hydroxychloroquine retinopathy. The possibility of vision loss prompted her to reconsider her prior decision to discontinue anticoagulation. This case demonstrates how anchoring bias may lead clinicians astray, and how the risk of blindness is a strong motivator for patients regarding anticoagulation.

A woman in her late 70s presented with an increased frequency of micturition, suprapubic pain and weight loss. She was found to be having advanced cancer of the urinary bladder, coupled with bilateral hydronephrosis.Whilst undergoing surgical intervention for the latter, she was incidentally found to be having hypercalcaemia. This was found to be paraneoplastic in nature, possibly due to elevated parathyroid hormone related peptide with no evidence of bone metastasis. The histology of the resected tumour revealed squamous and sarcomatoid differentiation. Her hypercalcaemia initially responded to intravenous fluids, and later on zolendronate,but the problem recurred again, with the response to a repeat dose of zolendronate and even denosumab being unsatisfactory. As a last resort cinacalcet was started, and although there was a good response to it, our patient sadly died a few weeks later.We believe our case to be the first case of hypercalcaemia associated with isolated bladder cancer which showed a successful response to cinacalcet.

Level 3 tracheal injury, characterised by complete laceration with oesophageal or mediastinal soft-tissue herniation, is conventionally indicated for surgical repair. We present the case of a woman in her 60s with level 3 tracheal injury in acute coronary syndrome who was treated successfully without surgery. Venoarterial extracorporeal membrane oxygenation (ECMO) was introduced during lung-rest management to avoid positive pressure ventilation, and percutaneous coronary angioplasty was performed. Venoarterial ECMO was switched to venovenous ECMO when the haemodynamics improved. The tracheal injury healed spontaneously in 10 days, and ECMO was withdrawn on day 15. The patient made full recovery and was discharged on day 51.

After interdisciplinary consent, a male patient in his 50s received endovascular treatment of an innocent supraophthalmic aneurysm of the internal carotid artery with a WEB (woven endo bridge) device and stenting. In the medical history, a migraine with aura was reported. A week after intervention, the patient developed an ipsilateral cluster-like headache responsive to classical interventions. Inflammation of the carotid wall near the stent was demonstrated to be associated with the headaches and was responsive to steroid treatment.The presented case demonstrates that postinterventional headache comprises more headache entities than the often reported migraine and tension-type headache with a considerable impact on the patients’ quality of life. The case supports the vascular hypothesis of cluster headache pathophysiology, potentially contradicting current models and assumptions.

Familial amyloidosis of the Finnish type (FAF) is a rare multisystemic disorder caused by mutations in the gelsolin gene. The clinical presentation is typically characterised by a triad of ophthalmic, neurological and dermatological findings. FAF has been reported in several countries, primarily in Finland and recently in Portugal. We report the first genetically confirmed cases of FAF from two unrelated families in our neuromuscular outpatient clinic. Gelsolin gene sequencing revealed the heterozygous gelsolin mutation (c.640G>A). The clinical features and the neurophysiological studies of two index patients and their relatives are presented. Obtaining an early diagnosis can be challenging, but FAF should be considered in the differential diagnosis of progressive bilateral facial neuropathy, even if there is no known Finnish ancestor.

Non-valvular atrial fibrillation (NVAF) is the most common cause of cardioembolic stroke. The left atrial appendage (LAA) is the major source of cardiac emboli in patients with NVAF. Anticoagulation (AC) is the standard of care for stroke prevention in atrial fibrillation (AF), but many patients are intolerant of AC. Surgical exclusion of the LAA may result in incomplete closure and is associated with an increased risk of embolism. We report a case of a woman in her 50s with a history of persistent AF, mitral valve prolapse s/p repair with surgical LAA exclusion, and multifocal haemorrhagic stroke presented for elective LAA closure who underwent a Watchman placement successfully. This case demonstrates that a percutaneous approach for occlusion of the LAA when surgical exclusion was incomplete may be feasible with appropriate planning. Clinical outcome data for this patient group are needed.

The literature on the anaesthetic management of a toddler presenting to the emergency department with an acute epidural haematoma with an uncorrected tetralogy of Fallot is sparse and management can be challenging. The main anaesthetic goals are to increase or maintain systemic vascular resistance and decrease pulmonary vascular resistance for cardiovascular stability, while maintaining cerebral perfusion pressure and decreasing intracranial pressure for cerebral protection. Thus, a balanced approach is desirable while managing such cases from emergency to the intensive care unit.

Steroids are commonly prescribed medications that have a wide range of adverse effects. Bradycardia is one of the rare but significant side effects of steroid use, and only a few cases have been reported with bradycardia as a side effect. In this report, we present a case of a woman in her early 50s who developed severe symptomatic sinus bradycardia following high-dose administration of intravenous hydrocortisone, initiated for acute exacerbation of Crohn’s disease. Her symptoms entirely resolved after discontinuation of the steroid. This case highlights the importance of obtaining baseline ECG and cardiac monitoring in patients treated with pulsed high-dose steroids.

An immunocompetent man in his 20s presented with a 24-hour history of severe odynophagia, nausea, vomiting and throat pain. Esophagogastroduodenoscopy (EGD) revealed severe esophagitis with ulcerated mucosa, exudative debris, haemorrhage and multiple erosions. Biopsy of the oesophageal tissue demonstrated marginated chromatin, multinucleated giant cells and molding of nuclei, consistent with herpes simplex virus esophagitis (HSE). Treatment with oral acyclovir led to the complete resolution of symptoms. The patient subsequently developed dysphagia again, 8 months later. EGD showed furrowing and concentric rings typical of eosinophilic esophagitis (EoE), a diagnosis confirmed by biopsy. Treatment with a proton pump inhibitor and swallowed topical corticosteroids led to symptomatic improvement. Thus, HSE can occur in immunocompetent hosts and on occasion, HSE may be a harbinger of EoE, as evidenced by our extensive literature review. Mechanical disruption of the mucosal barrier by viruses, facilitating food allergen penetration, and associated immunological signaling abnormalities may be responsible phenomena requiring further elucidation.

A male patient presented with a sudden visual decline in the right eye (OD). Fundus revealed bilateral vasculitis; OD also showed an occluded inferior retinal vein and a wedge-shaped retinal opacification of the inferior macula and nasal retina. Fluorescein angiography revealed occlusive retinal vasculitis, while optical coherence tomography showed paracentral acute middle maculopathy (PAMM) in the OD. A thorough systemic evaluation revealed hyperhomocysteinemia and a positive Mantoux test. A diagnosis of PAMM with occlusive retinal vasculitis in presumed intraocular tuberculosis and hyperhomocysteinemia was made. Retinal vasculitis improved with oral corticosteroid, intravitreal anti-vascular endothelial growth factor and laser photocoagulation. However, the patient declined antitubercular therapy despite recommendations. This unique report indicates that PAMM may complicate tubercular retinal vasculitis, especially in the presence of systemic hypercoagulable states.

Klebsiella invasive syndrome is a rare condition that typically presents as a liver abscess with metastatic infection, with mortality rates as high as 14% potentially due to diagnostic delay by clinicians. Here, we present a case of a woman in her 60s, who presented with symptoms and signs consistent with meningitis, imaging findings suggestive of possible leptomeningeal carcinomatosis a long with areas of lung consolidation and abdominal nodules and lymphadenopathy, presumably metastatic malignancy. We diagnosed Klebsiella invasive syndrome and treated it conservatively with medical management, including a long course of intravenous antibiotic therapy and supportive care. This is an infrequently encountered clinical entity with potentially fatal consequences, and we hope to add to the existing literature on the subject and drive home the point that it should be considered in the differential diagnoses in the appropriate clinical scenario.

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally invasive dermal sarcoma. The management is generally surgical, with wide local excision (WLE) forming the mainstay of treatment. Large abdominal wall defects are most aesthetically reconstructed using pedicled or free flaps; however, these require tumour-free surgical margins, and are off-set by donor site morbidity. We describe an alternative, aesthetic and low-morbidity technique for reconstruction of a subfascial defect following WLE of DFSP in a young woman in her early 20s, using two layers of a novel synthetic dermal matrix (NovoSorbBTM). To our knowledge, a double-layer reconstruction using an artificial dermal matrix has never been described for trunk reconstruction. We found that double-layer biodegradable temporising matrix can restore the inherent thickness and pliability of skin in a partial-thickness abdominal wall defect and offers improved durability and cosmesis compared with skin grafting or indeed single layer skin substitutes alone.

Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumour, and primary retroperitoneal PEComa is rarer still. Although pulmonary lymphangioleiomyomatosis (LAM), angiomyolipomas and clear cell ‘sugar’ tumours of the lung are well described, relatively little is known about other members of the PEComa family. We describe a case of an asymptomatic retroperitoneal PEComa, lymphangioleiomyoma type, which appeared in a previously healthy middle-aged woman as an incidental finding, in a CT scan performed in the context of spontaneous pneumothorax. The patient underwent surgical excision of the tumour and the histopathological and immunohistochemical analysis of the surgical specimen made the definitive diagnosis. Although rare, reports of isolated retroperitoneal lymphangioleiomyoma and primary retroperitoneal PEComas NOS (not otherwise specified) are described in the literature, normally associated with pulmonary LAM. The patient’s pulmonary imaging was normal. Short-term re-examination did not detect any recurrence. We also provide a literature review of this rare group of tumours.

A woman in her mid-60s presented to transient ischaemic attack (TIA) clinic with a 3-year history of intermittent sensory changes and white discolouration affecting the left side of her tongue. Following extensive investigation, a provisional diagnosis of posterior circulation TIA was made, and the patient was commenced on clopidogrel therapy. Despite anti-platelet treatment, she continued to have identical episodic symptoms. She was referred to the rheumatology team for assessment of possible underlying autoimmune pathology. On rheumatology assessment, the patient reported colour changes on the tongue, associated with numbness, followed by paraesthesia of the affected area. A comprehensive assessment excluded secondary causes and a diagnosis of primary Raynaud’s phenomenon of the tongue was made. The diagnosis of TIA was revoked. This case illustrates a rare presentation of a common condition and highlights the sensory symptoms which are associated with Raynaud’s phenomenon.

A woman in her 40s presented with a 3-month history of lower abdominal pain and intermenstrual bleeding. Ultrasound of the pelvis disclosed a 4 cm left adnexal mass. An MRI of the pelvis revealed a 2.2×3.6×2.4 cm solid, enhancing left ovarian mass. Due to high suspicion for malignancy, she underwent laparoscopic left salpingo-oophorectomy and resection of the tumour. Histopathology revealed papillary thyroid carcinoma in the background of struma ovarii as confirmed by thyroglobulin and thyroid transcription factor-1 positivity on immunohistochemistry. BRAF mutation analysis was negative. An ultrasound of the thyroid gland showed two low-risk nodules. An iodine-123 whole-body scan showed normal uptake in the thyroid gland. Thyroid-stimulating hormone (TSH) was 1.070 mcIU/mL (0.450–4.500), and thyroglobulin was 6.8 ng/mL (1.5–38.5). We risk-stratified this patient as low risk for recurrence. Risk stratification of malignant struma ovarii is essential to determine suitable thyroid targeting adjuvant therapy and reduce the risk of recurrence.

Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome. The constellation of findings of unprovoked fractures, hypophosphataemia, urinary phosphate wasting and a negative genetic evaluation suggest a TIO diagnosis. Tumours leading to TIO are often small and difficult to localise using standard imaging studies. The68Ga-DOTATATE CT/positron emission tomography, a somatostatin receptor imaging modality, is the radiographical study of choice for localisation. It is highly sensitive and specific since tumours that cause oncogenic osteomalacia have been shown to express somatostatin receptors. Complete surgical resection is the treatment of choice; however, it may not always be feasible. Burosumab, a human anti-fibroblast growth factor-23 monoclonal antibody, is a therapeutic option in cases of unresectable TIO to normalise phosphorus levels and improve fracture healing. Our patient was initiated on burosumab, which led to healing of his fractures and profound symptomatic improvement of his pain. TIO is often undiagnosed for many years, leading to significant patient morbidity.

Haematospermia, even though alarming, is usually benign and self-limiting, especially in a sexually active male. Nevertheless recurrent, refractory or painful haematospermia is troublesome and warrants thorough evaluation. In this context, we describe a rare case of recurrent haematospermia whereby evaluation revealed haemorrhage in seminal vesicle cysts and consequently established the aetiology of autosomal dominant polycystic kidney disease.

Enamel renal syndrome (ERS) due to loss of function (LOF) mutation of FAM20A gene typically consists of hypoplastic amelogenesis imperfecta (AI) and bilateral nephrolithiasis/nephrocalcinosis. Recent evidence suggests that FAM20A interacts with FAM20C and increases its activity; thus LOF mutation of FAM20A leads to impaired FAM20C action. FAM20C, a golgi casein kinase, phosphorylates fibroblast growth factor (FGF)-23, prevents its glycosylation and makes it more susceptible to degradation by furine proteases. Consequently, inactivating mutations of FAM20C lead to increased concentration of bioactive and intact FGF-23 in circulation and resultant hypophosphataemia. LOF mutation of FAM20A, thus, might also be associated with FGF-23-mediated hypophosphataemia; however, such an association has never been reported in the literature. We describe, for the first time, a triad of AI, bilateral nephrolithiasis and FGF-23-mediated hypophosphataemia in LOF mutation of FAM20A. We suggest that serum phosphate should be measured in all patients with ERS to avoid metabolic and skeletal complications of undiagnosed, hence untreated hypophosphataemia.

A woman in her early 30s presented herself with acute dyspnoea and elevated D-dimers 5 weeks after delivery of her second child. Echocardiographic findings showed signs of acute left ventricular failure, and an MRI confirmed a non-ischaemic dilated left heart failure compatible with peripartum cardiomyopathy. The antihormonal therapy with bromocriptine during 6 weeks and an intensive heart failure therapy led to an amelioration of the heart function within 3 years, but full recovery was not yet observed.

We present a case of Onyx embolisation of a renal pseudoaneurysm following partial nephrectomy with collecting system involvement with subsequent migration of Onyx into the renal collecting system resulting in renal obstruction. This occurred both immediately after embolisation and again 8 years after embolisation. Both cases required ureteroscopic surgical intervention. In the first instance, the pieces were removed using basket extraction. In the second, laser lithotripsy was used in addition to basket extraction. While there are a few cases of embolisation coils eroding into the renal collecting system, this is the second reported case of Onyx migration and the first where ureteroscopy with laser lithotripsy was used. The patient is doing well and undergoing surveillance ultrasounds to ensure there is no further Onyx migration. This may be a consideration for patients with pseudoaneurysm embolisation especially in the setting of prior collecting system damage.

We present a rare case of combined hepatocellular carcinoma-cholangiocarcinoma in a woman with a history of univentricular congenital heart disease requiring multiple corrective operations including Fontan procedure. During workup for elevated alpha fetal protein, a right hepatic lobe lesion was identified with biopsy showing poorly differentiated hepatocellular carcinoma. She underwent successful segment 5 liver resection. Final pathology demonstrated combined hepatocellular carcinoma-cholangiocarcinoma. She was treated with gemcitabine/oxaliplatin adjuvant chemotherapy and had no evidence of recurrent disease at her 12-month follow-up. To our knowledge, this is the first case reported in of successful treatment of this rare malignancy in the setting of Fontan-associated liver disease and highlights the importance of a robust screening protocol in this patient population. Semiannual screening for the development of primary liver malignancy should start by 10 years post-Fontan and continue until heart–liver transplantation may be performed. It is important to note that cirrhosis is not a pre-requisite for the development of hepatocellular carcinoma or cholangiocarcinoma in these patients.

Talon cusp is a rare dental anomaly which consists of a supernumerary projection from the cingulum towards the incisal edge of the tooth. The cusp is made up of enamel and dentin but pulpal tissue may or may not be present. The occurrence of mandibular talon cusp is a rare entity with only 58 cases reported until now. This article reports a case of talon cusp on a mandibular central incisor along with a literature review of all mandibular talon cusp reported in history.

A woman in her 70s with a history of Crohn’s disease presented to the emergency department with dyspnoea, cough and intermittent fevers. Evaluation revealed a pleural effusion with neutrophil predominance, and initial suspicion of infection prompted a short course of antibiotic therapy. However, the patient subsequently developed recurrent pleural effusion with eosinophilic predominance. Serological data confirmed a diagnosis of systemic lupus erythematosus (SLE) and the patient was started on appropriate treatment.This case presents an initial manifestation of eosinophilic-dominant pleural effusion in SLE. Current guidelines in treating pleural effusions do not explore rheumatological causes. However, we believe that our case demonstrates the value of a prompt investigation for rheumatological aetiologies in an otherwise unclassified eosinophilic-predominant pleural effusion. Such an investigation should include serological data as an important confirmatory marker for the diagnosis of SLE.