A univentricular heart : tricuspid atresia

A two-year-three-month-old boy was diagnosed with tricuspid atresia. This condition requires three surgical interventions, of which he has already had two. He has now presented to hospital with shortness of breath. This implies that he might now benefit from the third operation. Investigations, which were planned beforehand, will assess whether he is a candidate for this operation, that will improve his symptoms and hence his quality of life. Tricuspid atresia is the third most common cyanotic congenital heart disease. Patients with tricuspid atresia lack a tricuspid valve between the right atrium and right ventricle. Therefore, there is no right atrioventricular communication and as a result the right ventricle remains hypoplastic and undeveloped. These patients are thus left with only one functioning ventricle i.e.: the left ventricle, hence the term univentricular heart. Other defects that are present in patients with tricuspid atresia include an atrial septal defect (ASD), a ventricular septal defect (VSD) and possibly a patent ductus arteriosus (PDA).peer-reviewe

The subterranean sanctuary at Ħal Saflieni

The Hypogeum at Hal Saflieni is a unique structure on planet Earth; it is the only known megalithic monument which has been carved underground, and no parallels can be drawn with similar structures elsewhere. It not merely testifies to the precocious civilization of the Neolithic Maltese, but is a surviving model of the several Maltese megalithic structures above ground. Unlike the open stone circles outside the Maltese islands, such as Stonehenge, the Maltese megalithic repertoire is characterized by a massive enclosure housing a sanctuary; unlike the megalithic tombs enclosed and buried in supporting soil, the Maltese structures are entirely self-supporting and stand freely, without the surrounding matrix required elsewhere. They are therefore rightly classed as the earliest temples on the planet.peer-reviewe

Syndrome of inappropriate anti-diuretic hormone secretion (SIADH) and posterior cerebral artery ischaemic event : two uncommon complications following posterior fossa decompression

Neurosurgical procedures in cases of Type 1 Arnold Chiari Malformation (ACM) may result in a wide spectrum of complications. We report a case of a sixty-four year lady who underwent an elective posterior fossa decompression for Type 1 ACM. The procedure was complicated by syndrome of inappropriate anti-diuretic hormone secretion (SIADH) and an ischaemic cerebrovascular event affecting the posterior cerebral artery. The association of these complications with the procedure is rarely described in the literature. In spite of the poor prognosis associated with such complications, the patient made a relatively quick and uneventful recovery.peer-reviewe

A second vestigial umbilical vein : a case report

A healthy female infant was found to have two umbilical arteries and two umbilical veins at the cut surface of the cord at birth. Detailed inspection of the cord showed the second vein to represent a short segment vestigial vessel that, moreover, was not associated with any other congenital anomaly as is often found in infants with umbilical vein anomalies.peer-reviewe

A large pericardial effusion and bilateral pleural effusions as the initial manifestations of Familial Mediterranean Fever

Familial Mediterranean Fever (FMF) is a condition characterized by recurrent febrile poly-serositis. Typical presentations of the disease include episodes of fever, abdominal pain and joint pains. Chest pain is a less common presentation. We report a case of FMF which presented with a large pericardial effusion and bilateral pleural effusions in a lady who had no positive family history and negative genetic testing.peer-reviewe

A rare case of congenital pulmonary airway malformation presenting with chest pain and dyspnoea in an adult

A healthy 19-year-old man, who was a non-smoker, presented with a first episode of sudden onset, unprovoked dyspnoea and pleuritic chest pain. Chest X-ray showed a cystic abnormality in the right lung (Figure 1). Subsequently, a computed tomography scan of the thorax revealed large cysts in the right lower lobe (Figure 2), in keeping with a diagnosis of congenital pulmonary airway malformation (Stocker, 1994, 2002). The patient underwent a right lower lobectomy.peer-reviewe

Chronologic and dental ages of Maltese schoolchildren : a pilot study

Objectives: Dental ageing systems are useful for forensic, research and clinical purposes. As no data exists relating to the dental development of the Maltese population, we set up a pilot study to initiate formation of a set of tables pertaining to the dental development of Maltese schoolchildren. Methods: Panoramic radiographic records of 120 patients aged 11 to 14 years were sequentially collected from the records kept at the School Dental Clinic, Floriana and St Luke’s Hospital. These records were matched for age and sex. The calcification of the teeth was graded according to Nolla (1960) and the results obtained compared to Nolla’s tables to determine how closely the Maltese population conforms to these tables. Results: We found no significant difference between the estimated (dental) age and the chronological age of male schoolchildren. A significant difference existed for female schoolchildren. The dental age of the female schoolchildren was delayed when compared to that of male schoolchildren. Conclusion: Nolla’s tables require to be adjusted to take into account the variation in dental development of the Maltese population. Maltese schoolgirls exhibit slower dental development when compared to the figures given in the literature.peer-reviewe

Society for Endocrinology Endocrine Update 2019

A sixty-four-year old lady with a past medical history of total thyroidectomy for Graves’ disease and atrial fibrillation on amiodarone was referred to the endocrine out-patients in view of hypothyroidism. Since starting amiodarone, the TSH was noted to be elevated at 75 mU/l and the free T4 was 18 pmol/l. The patient complained of non-specific lethargy and was administered levothyroxine 50 mcg daily. Despite this her TSH remained elevated at 147 mU/l with a free T4 of 17.3 pmol/l and a free T3 of 2 pmol/l. TSH interference was ruled and a pituitary profile was normal. In the time of 2 years, despite gradual augmentation of her levothyroxine dose, the TSH remained elevated. At this point it was suspected that amiodarone might be causing type 1 5’ deiodinase enzyme inhibition leading to reduced peripheral conversion of T4 to T3. Hence the patient was commenced on a trial of liothyronine 12.5 mcg twice a day in addition to her levothyroxine. This eventually resulted in normalization of her thyroid function tests. Amiodarone induced hypothyroidism highlights the importance of life long thyroid function monitoring (free T3, free T4 and TSH) in patients on amiodarone. This case of amiodarone-induced hypothyroidism also reveals another possible indication for levothyroxine/liothyronine combination therapy.peer-reviewe

Sodium-glucose co-transporter 2 (SGLT2) inhibitors

Type 2 diabetes mellitus is a progressive metabolic disorder. Marked hyperglycaemia leads to serious vascular complications. Hence, addressing this modifiable risk factor is of paramount importance. Sodium-glucose co-transporter 2 (SGLT2) inhibitors represent a relatively new class of antidiabetic agents. They offer an intermediate glucose-lowering effect and through other pleiotropic effects provide cardiac and renal benefits. This review focuses on the mechanism of action, benefits and adverse effects of SGLT2 inhibitors. The authors also delineate the ideal type 2 diabetic candidate to receive SGLT2 inhibitors. This is critical as SGLT2 inhibitors should not be used in a ‘one-size-fits-all approach’ but their use should be individualized based on certain patient characteristics. This patient-centred approach aims at maximizing the benefits and reduce the risks associated with SGLT2 inhibitors.peer-reviewe

Diagnosis and growth dynamics of adrenal incidentalomas : a 6-month retrospective analysis

INTRODUCTION: Adrenal incidentalomas are adrenal masses discovered incidentally on imaging studies originally not performed for suspected adrenal disease.AIM: To characterise a cohort of adrenal incidentalomas found on CT imaging the adrenal region.METHODS: This was a retrospective analysis, taking into account all the adrenal incidentalomas discovered on CT between July and December 2014 at Mater Dei Hospital. Only lesion greater than 1cm were included in the study. These were then classified according to their radiological features. Previous CT scans and any CT scans done after the study period were also reviewed to establish any change is size of the lesions.RESULTS: A total of 9100 CT scans were reviewed and adrenal incidentalomas were identified in 296 patients. 216 incidentalomas could be adequately classified and included in the study; 80.1% were confirmed adenomas, 12.9% metastasis, 5.6% myelolipomas and 1.3% ganglioneuromas. 49.1% of patients with an adenoma were males as opposed to 71.4% in the metastasis group. Bilateral lesions were commoner in the metastasis group (9% in adenoma vs 18% in metastasis group). Longest median radiological diameter was 18mm (IQR 14.0-24.0) in the adenoma group and 26.0mm (IQR 16.0-36.0) in the metastasis group (P<0.001). Median follow up in the adenoma group was longer in the adenoma group 20.9 months (IQR 4.5-39.0) vs 11.2 months (IQR 0-29.3) in the metastasis group (P<0.001). Median change in size was also statistically significant: 0.0mm (IQR -1-0.8) in the adenoma as compared to 22.5mm (IQR 12.5-30) in the metastasis group (P<0.001).CONCLUSION: This study continues to confirm that adrenal adenomas are the commonest adrenal lesion encountered in clinical practice and the majority, by far remain stable in size over time.peer-reviewe