Anterior mediastinal tracheostomy in a child with Hurler syndrome

Shutoh Takashi, Funai Yusuke, Hori Kotaro, Waki Sayaka, Mori Takashi

doi:10.3918/jsicm.30_235

<p>We hereby report the case of a 10-year-old boy with Hurler syndrome who underwent an anterior mediastinal tracheostomy due to severe limitation in performing cervical retroflexion. He was diagnosed with Hurler syndrome during his infancy period, and he had undergone a laminectomy for cervical spondylosis at the age of 4 years. Before his admission to our hospital, the patient was intubated at another hospital due to ARDS. Thence, he was referred to our hospital for further intensive care. The pneumonia improved after intensive care; however, he persistently had difficulties weaning from the ventilator and thus, needed a tracheostomy. However, the tracheostomy was considered impossible due to the fact that he suffered from severe cervical retroflexion restriction. Consequently, an anterior mediastinal tracheostomy was planned. Since this procedure is rarely performed in children, and because he had serious comorbidities, a meeting was organized with specialists from different disciplines to closely discuss the perioperative management. We particularly discussed about the surgical positioning, the intubation method, the feasibility of extracorporeal circulation, and how to deal with the risk of brachiocephalic arteriovenous dissection. The surgery was completed uneventfully, and his postoperative course was relatively good without any severe complications. He could be safely managed through multidisciplinary treatment in collaboration with various professionals.</p>

Anterior mediastinal tracheostomy in a child with Hurler syndrome

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