Tumoral Calcium Pyrophosphate Dihydrate Deposition Disease of the Ligamentum Flavum

Abstract

AbstractOBJECTIVECalcium pyrophosphate dihydrate (CPPD) deposition disease (CPPDD), also known as pseudogout, is rarely known to affect the spine. The purpose of this article is to report our experience with six cases involving massive focal deposition of CPPD crystals in the ligamentum flavum.METHODSBetween January 1998 and June 2002, we treated six patients with CPPDD involving the ligamentum flavum of the cervical and thoracic spine. Their ages ranged from 45 to 70 years. There were five female patients and one male patient. The cervical spine was involved in two cases and the thoracic spine in four. All except one patient presented with an insidious onset of myelopathy. The remaining patient presented with paraplegia after trauma. None of the patients exhibited any systemic features of CPPDD or other metabolic conditions that can lead to CPPD deposition. Plain x-rays often yielded inconclusive results. Computed tomography and magnetic resonance imaging were useful in confirming the diagnoses. Decompressive laminectomy, with removal of the ossified ligamenta flava, was performed for all patients. Polarized-light microscopic examinations of the excised ligamenta flava revealed the characteristic rod-shaped, birefringent crystals.RESULTSFive of the six patients experienced significant improvements in their myelopathic symptoms after surgery. The remaining patient experienced improvements in sensations but no appreciable improvement in motor power. During the follow-up periods, which ranged from 7 months to 3 years, none of the patients presented with a recurrence of CPPD crystal deposition at the previously treated level. However, one patient who exhibited improvement after surgery presented 2 years later with a recurrence of myelopathic features attributable to ossification of the ligamentum flavum at a new level.CONCLUSIONTumoral CPPDD of the ligamentum flavum is rare. It commonly occurs among middle-age or elderly female patients and presents with progressive myelopathy. Computed tomography and magnetic resonance imaging are complementary in the diagnosis of this condition. Surgery, if performed early, leads to good improvement. However, long-term follow-up monitoring of these patients is necessary, because surgery provides only symptomatic relief and does not treat the underlying disease. With the increasing availability of magnetic resonance imaging, ossification of the ligamentum flavum is being more frequently recognized. In every case of ossified ligamentum flavum, the excised specimen should be examined with polarized-light microscopy. We think that this simple, inexpensive method will lead to the recognition of more cases of spinal CPPDD.