Currarino-Silverman syndrome: diagnosis and treatment of rare chest wall deformity, a case series.

ABSTRACT

BACKGROUND: Currarino-Silverman (CS) syndrome is an extremely rare congenital deformity of the anterior chest wall. The syndrome is often combined with congenital heart defects and spinal abnormalities. As of currently, there is a lack of definite description in the literature about this type of pectus deformity. Typically, patients do not require surgical intervention for medical reasons, and the correction is usually only for cosmetic purposes. The purpose of this study was to demonstrate surgical intervention for CS syndrome at a tertiary care facility, and to summarize the available literature. METHODS: Patients with CS syndrome were retrospectively reviewed from a period of June 2012 to August 2019. An extensive literature search for "Currarino-Silverman syndrome," "pouter pigeon chest," "chondromanubrial deformity," "type 2 pectus carinatum" and "pectus arcuatum" was performed. RESULTS: Four clinical cases of CS syndrome are presented, two of which were symptomatic and corrected. The procedure of choice was the modified Ravitch-type thoracoplasty with double osteotomy and implantation of support plates. CONCLUSIONS: There is no clear definition of CS syndrome in the literature. Correct and uniform classification plays a crucial role in the surgical treatment of this pathology. Due to the extreme rarity of the disease, challenging deformity, and variable anatomy of the fused sternum, there are no clear guidelines in treatment approaches. The correction is mostly pursued only for cosmetic results, and the best surgical option for CS syndrome remains the relatively aggressive Ravitch-type procedure with multi-level wedge osteotomy.