Surgical treatment of a 36-year-old patient with asphyxiating thoracic dysplasia

Abstract

Asphyxiating thoracic dysplasia is a rare and dangerous genetic disease. Many children with this disease die early in life of severe hypoxia, and it is extremely rare that they survive to adulthood. We recently treated a 36-year-old patient who had asphyxiating thoracic dysplasia with a special surgical method and achieved satisfactory results. A review of the literature showed that this patient is the oldest surviving person with this condition.

Novel median thoracic expansion for asphyxiating thoracic dystrophy

“…Lesions of ATD involve multiple organs, but the main manifestation is narrow chest [ 3 , 4 ]. The only effective way to treat it is operation.…”

“…The main purpose of the operation is to increase the thorax volume. There are many methods reported in the literature, which can be summarized into three categories: median thoracic expansion (MTE) [ 1 ], lateral thoracic expansion (LTE) [ 2 ] and orthopaedic surgery [ 3 , 4 ]. Different authors have used these methods to treat ATD, but no one has discussed the indications of these methods [ 1–4 ].…”

Bilateral correction of asphyxiating thoracic dystrophy

“…Asphyxiating thoracic dystrophy (ATD) is a rare autosomal recessive disease reported by Jeune in 1955 [ 1 ]. It’s narrow thorax is the main factor threatening the life of patients, and operation is recognized as the only effective method of treatment [ 2 , 3 ].…”

“…It’s narrow thorax is the main factor threatening the life of patients, and operation is recognized as the only effective method of treatment [ 2 , 3 ]. Several operations have been used in clinic in the past [ 1–4 ]. However, they all have disadvantages.…”

Surgical treatment of asphyxiating thoracic dystrophy with median thoracic expansion and Nuss procedure

“…Asphyxiating thoracic dystrophy (ATD), also known as Jenue syndrome, was first reported in 1955 [1,2] . This disease is an autosomal recessive disease, which is extremely rare and dangerous.…”