Prophylactic thyroidectomy in children with multiple endocrine neoplasia type 2.

BACKGROUND: In patients with multiple endocrine neoplasia type 2 (MEN2) syndrome, genetic testing offers early diagnosis, stratifies the risk of developing medullary thyroid cancer (MTC) and informs the timing of thyroidectomy. The efficacy of treatment, which depends on timely and safe surgery, is not well established. METHODS: This was a retrospective review of diagnostic and clinicopathological outcomes of prophylactic thyroidectomy in children with MEN2 between 1995 and 2013 in the UK. American Thyroid Association (ATA) 2009 guidelines were used as a benchmark for adequate treatment. RESULTS: Seventy-nine children from 16 centres underwent total thyroidectomy. Thirty-eight patients (48 per cent) underwent genetic testing and 36 (46 per cent) had an operation performed above the age recommended by the ATA 2009 guidelines; pathology showed MTC in 30 patients (38 per cent). Late surgery, above-normal preoperative calcitonin level and MTC on pathology correlated with late genetic testing. Twenty-five children had lymphadenectomy; these patients had more parathyroid glands excised (mean difference 0·61, 95 per cent c.i. 0·24 to 0·98; P = 0·001), and were more likely to have hypocalcaemia requiring medication (relative risk (RR) 3·12, 95 per cent c.i. 1·54 to 6·32; P = 0·002) and permanent hypoparathyroidism (RR 3·24, 1·29 to 8·11; P = 0·010) compared with those who underwent total thyroidectomy alone. Age did not influence the development of complications. CONCLUSION: Late genetic testing may preclude age-appropriate surgery, increasing the risk of operating when MTC has already developed. Early genetic testing and age-appropriate surgery may help avoid unnecessary lymphadenectomy and improve outcomes.

Coil migration--a rare complication of endovascular exclusion of visceral artery pseudoaneurysms and aneurysms.

INTRODUCTION: We describe a case of metallic, angiographic coil migration, following radiological exclusion of a gastroduodenal artery pseudoaneurysm secondary to chronic pancreatitis. PATIENTS AND METHODS: A 55-year-old man presented to the out-patient clinic with chronic, intermittent, post-prandial, abdominal pain, associated with nausea, vomiting and weight loss. He was known to have chronic pancreatitis and liver disease secondary to alcohol abuse and previously underwent angiographic exclusion of a gastroduodenal artery pseudoaneurysm. During subsequent radiological and endoscopic investigation, an endovascular coil was discovered in the gastric pylorus, associated with ulceration and cavitation. This patient was managed conservatively and enterally fed via naso-jejunal catheter endoscopically placed past the site of the migrated coil. This patient is currently awaiting biliary bypass surgery for chronic pancreatitis, and definitive coil removal will occur concurrently. CONCLUSIONS: Literature review reveals that this report is only the eighth to describe coil migration following embolisation of a visceral artery pseudoaneurysm or aneurysm. Endovascular embolisation of pseudoaneurysms and aneurysms is generally safe and effective. More common complications of visceral artery embolisation include rebleeding, pseudoaneurysm reformation and pancreatitis.

Adrenal suppression following intralesional corticosteroids for periocular haemangiomas.

BACKGROUND/AIMS: Treatment with intralesional triamcinolone/betamethasone is recommended for infantile sight-threatening periocular haemangiomas. This study investigates the endocrine and weight changes in 15 infants undergoing therapy over 12 years. METHODS: 15 infants, median age 19 weeks (range 10-56) receiving intra/perilesional triamcinolone/betamethasone underwent serial measurement of weight, early morning serum cortisol and adrenocorticotropic hormone (ACTH) before and after injection. RESULTS: Cortisol fell from a median (range) of 383 (112-594) to 28 (<10-506) nmol/l (p=0.005) and ACTH from 26 (14-134) to 9 (5-20) ng/l (p=0.05) from before injection to 4 weeks after treatment. Prolonged adrenal suppression occurred in 13 out of 15 cases with time to recovery being 19.5 (4-65) weeks. Failure to gain weight appropriately was observed in 14 infants but recovered once normal adrenal function was re-established. CONCLUSION: Prolonged adrenal suppression following triamcinolone/betamethasone injection for periocular haemangiomas is common and associated with faltering weight gain.