Spontaneous peripheral nerve palsy with hourglass-like fascicular constriction in the upper extremity

Spontaneous paralysis from hourglass-like fascicular constriction of peripheral nerves is rare, its clinical manifestations are not well documented, and its pathogenesis remains unknown. The unclear origin of this disorder and difficulty in diagnosis result in its uncertain management. The authors sought to gain a more thorough understanding of this condition through describing the anatomy, clinical features, etiology, and treatment of hourglass-like constriction.

The authors retrospectively reviewed 20 patients (22 nerves) with hourglass-like constriction. The patients' clinical information was reviewed. Preoperative sonographic assessment and electrophysiological examination of involved nerves were performed. Surgical treatments included interfascicular neurolysis and neurorrhaphy. Samples of tissue subjected to resected constriction were sent for pathological analysis. The patients had regular face-to-face follow-up visits.

Acute pain was always the first symptom and was followed by paralysis. Paralysis progression was rapid and serious. Surgical exploration indicated an hourglass-like constricted segment completely unrelated to the compressive structures. Electrophysiological analysis showed severe denervation, and histopathological examination showed inflammatory cell infiltration, demyelination, and reduction of nerve fibers.

Hourglass-like fascicular constrictive neuropathy has an integrative effect from multiple different mechanisms. Surgical intervention is beneficial for selected patients who do not recover in a timely fashion and have hourglass-like lesions confirmed by preoperative ultrasound imaging. The authors recommend that early surgical intervention of the nerve be offered to patients who do not show any signs of recovery 3 months after onset. Both interfascicular neurolysis and neurorrhaphy are effective treatment methods. Mild to moderate constriction can usually be treated successfully by interfascicular neurolysis alone, whereas more advanced lesions with loss of fascicle continuity (severe constriction) may be best treated with resection and direct neurorrhaphy.