Case: We report the first case of a 21-year-old male patient with macrodystrophia lipomatosa (MDL), with local gigantism involving the thoracic spine and progressively worsening neurodeficit. Imaging studies revealed fatty infiltration and hypertrophied intercostal nerves, dextroscoliosis, osseous hypertrophy between C4 and T2, and severe canal stenosis at the T4 to T5 level secondary to lamino-facetal hypertrophy. He underwent debulking of the lesion and posterior instrumented decompression in a staged manner. His neurodeficit improved postoperatively and was ambulant without support at the end of the 2-year follow-up.
Conclusion: Surgery in patients with MDL is technically challenging and fraught with complications such as neurological deficit and significant blood loss.
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