A Report of Congestive Myelopathy as a Result of Chronic Chylous Reflux Syndrome

Dear Editor, We would like to share an interesting case of congestive myelopathy involving a 26-year-old female with chronic gy-necological chylous reflux disease who presented with bilateral lower extremities weakness, decreased sensation, and urinary incontinence of 2 months duration. A medical review revealed chronic vaginal discharge and lower-extremity swelling due to chronic chylous reflux disease. Upon examination, the patient had normal muscle strength in the upper extremities , motor power grades of 4 in the left lower extremity (LLE) and 1 in the right lower extremity (RLE), and loss of rectal tone. Sensory examination revealed decreased touch and vi-bratory sensation in both lower extremities. All cranial nerve and cerebellar functions were intact. Spinal MRI revealed T2-weighted hyperintense signals and atrophy of the thoracic cord with enhancing curvilinear T2-weighted hypointense signals within the thecal sac and flow voids anterior and posterior to the conus medullaris (Fig. 1). The findings were suggestive of a dural arteriovenous malformation (AVM). Surprisingly , subsequent spinal angiography did not reveal any evidence of either a dural AVM or a fistula. We therefore diagnosed this patient with congestive myelopathy secondary to venous drainage dysfunction as a result of chylous reflux syndrome. Primary chylous reflux syndrome is a rare lymphatic dis order that is characterized by retrograde flow of chyle from its normal route. Hypothesized pathophysiologies include an incompetent lymphatic valvular system, lymphatic hypopla-sia, and dilated incompetent megalymphatics. However, the definite pathophysiology remains unclear. Approximately 20 cases of primary chylous reflux syndrome have been reported during the past century. The common manifestations are lymphedema, chyluria, and chylous genital discharge. Some patients may experience complications such as chylo-thorax, chyloperitoneum, and chylopericardium. 3 However, neurological deficits have never been reported in association with this condition. This patient had been diagnosed with primary chylous re-flux syndrome as a young child, and was conservatively managed with dietary control. At the age of 26 years she gradually developed venous congestive myelopathy or Foix-Alajoua-nine syndrome. Given that the most common cause of ve-nous congestive myelopathy is spinal dural arteriovenous fistula, extensive investigations including MRI/MRA and spinal angiography were conducted. 4 The results were negative for concurrent vascular malformations. To date, only 14 cases of venous congestive myelopathy without concurrent vascular malformation have been reported, 5 none of which had underlying chylous reflux syndrome. Chyle is normally absorbed through the lacteals, drains into the thoracic duct, and empties into the venous system. In patients with …