[Prophylactic total thyroidectomy in childhood for multiple endocrine neoplasia type 2A: preliminary results]

Evaluation of prophylactic total thyroidectomy in childhood in case of MEN2A gene carriership.

Retrospective.

Prophylactic thyroidectomy was performed in 14 MEN2A gene carriers (7 boys, 7 girls; median age 9.1 year (range: 4.8-14.7)), in June 1993-July 1997 at the department Pediatric Surgery of the Wilhelmina Children's Hospital in Utrecht, the Netherlands. Median time between genetic investigation and operation was 5.5 months (range: 2-35). Lymph node dissection was not performed. The parathyroids were identified and left untouched as far as possible, autotransplantation was performed twice because of doubt about viability. Outpatient follow-up took place every 3-6 months.

One patient (13.4 year) showed macroscopic, the other 13 microscopic multifocal medullary thyroid carcinoma, 11 bilateral and 3 unilateral. In 1 child (6.2 year) neuroinvasive growth existed already. Surgical sections were free of tumour. After the operation temporary hoarseness occurred once, temporary hypocalcaemia three times and permanent hypoparathyroidism twice; after autotransplantation no hypocalcaemia occurred. Median follow-up was 3.2 year (range: 1 month-4.0 year). Mild psychological problems were observed in 4 patients, psychiatric problems in 1.

Prophylactic total thyroidectomy during the first decade is recommended. Additional lymph node dissection and total parathyroidectomy are unnecessary than. In order to prevent postoperative hypoparathyroidism, autotransplantation of at least one parathyroid is advisable.