Nutcracker phenomenon with a daily persistent headache as the primary symptom: Case series and a proposed pathogenesis model based on a novel MRI technique to evaluate for spinal epidural venous congestion

Daily persistent headache with nutcracker physiology and spinal epidural venous congestion: Treatment with lumbar vein embolization

“…A recent study that analyzed trMRA centered on the L2LV in patients with NDPH has demonstrated that there is a potential association between NCP and retrograde L2LV flow and EVP congestion [7] . This is similar to the pathophysiology implicated to cause headaches and myelopathies with the Queckenstedt maneuver or IVC and renal vein occlusion [10 , 11] .…”

“…Recently, Rozen et al. published a case series which demonstrates that 8/12 (67%) patients with new daily persistent headache (NDPH) had LRV compression, lumbar vein (LV) dilatation, and early spinal epidural venous plexus (EVP) enhancement on MRI [7] . They utilized contrast enhanced time-resolved magnetic resonance angiography (trMRA) in patients with NDPH to demonstrate that NCP can cause retrograde left second lumbar vein (L2LV) flow and secondary EVP congestion [7] .…”

“…published a case series which demonstrates that 8/12 (67%) patients with new daily persistent headache (NDPH) had LRV compression, lumbar vein (LV) dilatation, and early spinal epidural venous plexus (EVP) enhancement on MRI [7] . They utilized contrast enhanced time-resolved magnetic resonance angiography (trMRA) in patients with NDPH to demonstrate that NCP can cause retrograde left second lumbar vein (L2LV) flow and secondary EVP congestion [7] . The authors hypothesized that the EVP congestion causes a subsequent congestion of the cerebral venous system leading to elevation of cerebrospinal fluid (CSF) pressure which, in turn, results in the development of refractory headaches.…”

Update in the understanding of new daily persistent headache

“…Low-pressure headache due to CSF-venous fistula may resemble typical NDPH presentations, and surgical ligation completely resolved the headache in this case (47). Even more rarely, two independent groups reported daily-persistent headaches secondary to nutcracker syndrome – aortomesenteric compression of the left renal vein (48,49). One patient had nearly complete resolution of headache symptoms after aortomesenteric decompression (48), and six out of eight patients had immediate pain resolution after CSF volume removal (49).…”

“…Even more rarely, two independent groups reported daily-persistent headaches secondary to nutcracker syndrome – aortomesenteric compression of the left renal vein (48,49). One patient had nearly complete resolution of headache symptoms after aortomesenteric decompression (48), and six out of eight patients had immediate pain resolution after CSF volume removal (49). Notably, none of these eight patients had elevated intracranial pressure at baseline.…”