Chylous Cardiac Tamponade with Chylothoraces Secondary to Hodgkin’s Lymphoma: Octreotide in Conjuncture with Standard of Care Dietary Fat Restriction

Chylous effusions are a well-known complication from a variety of etiologies including trauma, malignancies, and anatomic defects, with the most common location being in the pleural space. A pericardial chylous effusion (chylopericardium) is uncommon, and a chylopericardium with concomitant bilateral chylous pleural effusions (chylothoraces) has only been reported in less than a handful of case reports. Our patient presented with bilateral chylothoraces and a chylopericardium with tamponade physiology secondary to Hodgkin’s Lymphoma. In this article, we discuss our treatment of this patient with the somatostatin analogue octreotide, as well as the standard of care dietary fat restriction, in order to control these effusions until the patient’s chemotherapy took effect.

Partial diazoxide responsiveness in a neonate with hyperinsulinism due to homozygous ABCC8 mutation

Summary We report a case of partial diazoxide responsiveness in a child with severe congenital hyperinsulinaemic hypoglycaemia (CHI) due to a homozygous ABCC8 mutation. A term baby, with birth weight 3.8 kg, born to consanguineous parents presented on day 1 of life with hypoglycaemia. Hypoglycaemia screen confirmed CHI. Diazoxide was commenced on day 7 due to ongoing elevated glucose requirements (15 mg/kg/min), but despite escalation to a maximum dose (15 mg/kg/day), intravenous (i.v.) glucose requirement remained high (13 mg/kg/min). Genetic testing demonstrated a homozygous ABCC8 splicing mutation (c.2041-1G>C), consistent with a diffuse form of CHI. Diazoxide treatment was therefore stopped and subcutaneous (s.c.) octreotide infusion commenced. Despite this, s.c. glucagon and i.v. glucose were required to prevent hypoglycaemia. A trial of sirolimus and near-total pancreatectomy were considered, however due to the significant morbidity potentially associated with these, a further trial of diazoxide was commenced at 1.5 months of age. At a dose of 10 mg/kg/day of diazoxide and 40 µg/kg/day of octreotide, both i.v. glucose and s.c. glucagon were stopped as normoglycaemia was achieved. CHI due to homozygous ABCC8 mutation poses management difficulties if the somatostatin analogue octreotide is insufficient to prevent hypoglycaemia. Diazoxide unresponsiveness is often thought to be a hallmark of recessively inherited ABCC8 mutations. This patient was initially thought to be non-responsive, but this case highlights that a further trial of diazoxide is warranted, where other available treatments are associated with significant risk of morbidity. Learning points: Homozygous ABCC8 mutations are commonly thought to cause diazoxide non-responsive hyperinsulinaemic hypoglycaemia. This case highlights that partial diazoxide responsiveness in homozygous ABCC8 mutations may be present. Trial of diazoxide treatment in combination with octreotide is warranted prior to considering alternative treatments, such as sirolimus or near-total pancreatectomy, which are associated with more significant side effects.

The use of the somatostatin analogue octreotide for the conservative management of symptomatic cholecystocolonic fistula: a case report

An 84-year-old woman presented with acute worsening of diarrhoea for a few weeks, with a background of chronic diarrhoea over the past 12 months accompanied by weight loss. Computed tomography during this admission revealed air in the biliary tree and resolution of gallstones in keeping with a cholecystocolonic fistula. Owing to her comorbidities, surgical management was deemed not to be the best option. She was trialled on octreotide, a somatostatin analogue, which effectively resolved her symptoms. This case presents an effective and novel method of managing cholecystocolonic fistulas conservatively in a patient where medical therapy is the ceiling of care.

Resistant Paediatric Somatotropinomas due to AIP Mutations: Role of Pegvisomant

Background: Somatotropinomas are rare in childhood and frequently associated with genetic mutations. AIP mutations are found in 20–25% cases and cause aggressive somatotropinomas, often resistant to somatostatin analogues. Aims: To assess responses to multimodal therapy including pegvisomant in 2 children with sporadic somatotropinomas due to AIP mutations. Case Description: We report 2 children, a boy aged 13 and a girl aged 10, with rapid growth, visual impairment, and growth hormone hypersecretion. Magnetic resonance imaging confirmed a pituitary macroadenoma with parasellar extension in both. Despite multiple surgical attempts to debulk tumour mass, residual tumour persisted. Genetic analysis showed two different AIP mutations (patient 1: c.562delC [p.Arg188Glyfs*8]; patient 2: c.140_ 163del24 [p.Gly47_Arg54del8]). They were initially treated with a long-acting somatostatin analogue (octreotide LAR 30 mg/month) and cabergoline as a dopamine agonist, with the later addition of pegvisomant titrated up to 20 mg/day and with radiotherapy for long-term control. Somatostatin analogue was ceased due to patient intolerance and lack of control. Patient 1 had normalization of insulin-like growth factor-1 (IGF-1) after 5 months of combined therapy with pegvisomant and cabergoline. For patient 2, normalization of IGF-1 was achieved after 2 months of cabergoline and pegvisomant. Conclusion: AIP-associated tumours can be resistant to management with somatostatin analogues. Pegvisomant can safely be used, to normalize IGF-1 levels and help control disease.

Glucagonoma-induced acute heart failure

Summary Neuroendocrine tumours (NETs) represent a broad spectrum of tumours, of which the serotonin-producing carcinoid is the most common and has been shown to cause right ventricular heart failure. However, an association between heart failure and NETs other than carcinoid has not been established so far. In this case report, we describe a 51-year-old patient with a glucagon-producing NET of the pancreas who developed acute heart failure and even cardiogenic shock despite therapy. Heart failure eventually regressed after initialising i.v. treatment with the somatostatin analogue octreotide. Chromogranin A as a tumour marker was shown to be significantly elevated, and it decreased with clinical improvement of the patient. The effects of long-time stimulation of glucagon on the myocardium have not been studied yet; however, sarcoplasmic reticulum calcium leak can be discussed as a possible mechanism for glucagon-induced heart failure. Learning points Glucagonoma can be a cause for heart failure. i.v. infusion of octreotide can be successfully used to treat glucagonoma-induced acute heart failure. We suggest that cardiac function should be monitored in all NET patients.