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Coffin-Lowry syndrome (CLS) is a rare X-linked disorder marked by intellectual disability, craniofacial and skeletal anomalies, and progressive spinal deformities like scoliosis and hyperlordosis. Corrective posterior spinal fusion (PSF) surgery is often required. Mesocardia, a congenital anomaly where the heart is positioned centrally in the thorax, may affect perioperative hemodynamics, particularly in the prone surgical position. This report presents a case illustrating the perioperative challenges of PSF in a patient with both CLS and mesocardia.

A 17-year-old male with CLS, mesocardia, and severe scoliosis underwent PSF (T2-S2). During prone positioning, the patient experienced a gradual hemodynamic collapse, characterized by a decrease in oxygen saturation and a marked drop in both pulse rate and blood pressure. Repositioning to the supine position led to immediate recovery. Imaging ruled out pulmonary or vascular obstruction, pneumothorax, and embolism. The event was attributed to mechanical cardiac compression related to syndromic soft tissue laxity, thoracic deformity, mesocardia, and posterior soft-tissue release. Surgery was completed using a modified lateral oblique prone position, maintaining hemodynamic stability.

This case illustrates the importance of anticipating cardiopulmonary compromise due to anatomical and positional factors and that personalized surgical planning and modified positioning can be crucial in complex spinal deformity surgery in syndromic patients with thoracic abnormalities.