gms | German Medical Science

Objectives: Marfan Syndrome (MS) is a connective tissue disorder which is associated with several musculoskeletal issues, in particular tall stature, scoliosis, genua valga and pes planus. Surgery may improve quality of life of affected individuals, but presumably is associated with increased complication rates. However, studies investigating the outcome of surgical treatment are scarce.

Methods: A retrospective chart review of 63 patients (39 female, 24 male) with MS who presented at our pediatric orthopedic department between 2012 and 2022 was conducted. Distribution of surgically treated musculoskeletal conditions, number of surgeries and complication rates were evaluated. The mean follow-up time was 4.1 (1.0–7.8) years.

Results and conclusion: Mean age at initial consultation at our department was 10.2 (0.7–24.3) years. Mean age at the time of last follow-up was 13.0 (1.8–27.4) years. Surgery was performed in 31 (49.2%) patients. Mean age at first surgery was 12.2 (5.9–20.6) years. Bilateral epiphysiodesis of the distal femur and proximal tibia for reduction of excessive height was performed in 11 patients (17.5%). Foot surgery was performed in 10 patients (15.9%) to correct pes planus, of whom 6 patients with flexible flatfeet received subtalar arthroereisis. In 4 patients corrective osteotomy of the foot was performed. Surgical correction of lower limb malalignment was performed in 5 patients (9.5%). 4 of whom received temporary hemiepiphysiodesis to correct valgus malalignment. In 1 of 5 patients correction osteotomy was conducted for varus malalignment. Spinal fusion was performed in 3 of 63 patients (4.8%) presenting with severe scoliosis. Nuss procedure for correction of pectus excavatum was performed in 1 patient (1.6%). The overall complication rate was 12.9%, with 4 of 31 suffering complications. In 2 patients insufficient correction of pes planus was observed. 1 patient suffered of rod breakage after spinal fusion, requiring revision surgery. 1 patient presented with unilateral valgus malalignment after epiphysiodesis, which was solved by temporary hemiepiphysiodesis. No cardiovascular complications were observed perioperatively.

The incidence of surgical treatment of musculoskeletal conditions related to MS is high, with half of all patients requesting surgical treatment. However, selection bias of the study group has to be taken into account. There appears to be high demand for surgical treatment of tall stature. Complication rates seem to be reasonable, and there does not seem to be an increased risk for perioperative cardiovascular complications.