Abstract
Background:Idiopathic hemorrhage in a syrinx is a rare entity known as Gowers’ intrasyringeal hemorrhage. Bleeding confined to the syrinx cavity causes severe, sometimes acute, neurological deficits. We report a case of intrasyringeal hemorrhage into a preexisting lumbosacral syrinx associated with Chiari type I malformation.Case Description:A 39-year-old female with Noonan syndrome underwent foramen magnum decompression and a cervical syrinx-subarachnoid shunt for Chiari type I malformation-associated syringomyelia 7 years ago. She presented progressive gait deterioration and acute urinary dysfunction, indicating conus medullaris syndrome. Initial magnetic resonance imaging revealed massive hemorrhage in the intrasyringeal cavity of the conus medullaris. The patient underwent surgical removal of the intrasyringeal hematoma and her neurological symptoms improved postoperatively.Conclusion:Although Gowers’ intrasyringeal hemorrhage is rare, this entity should be taken into consideration in patients with syringomyelia showing acute neurological deterioration.
Highlights
Idiopathic hemorrhage in a syrinx is a rare entity known as Gowers’ intrasyringeal hemorrhage
Intrasyringeal hemorrhage was first described by Gowers in 1904, and was named Gowers’ syringeal hemorrhage by Wilson in 1955.[1]. The hemorrhages have mostly been observed in syringomyelic cavities, and patients present with a sudden onset, and/or acute and rapid worsening, of pain
Since increased intrasyringeal pressure caused by hemorrhage may result in irreversible neurological sequelae, it is important not to overlook the symptoms caused by intrasyringeal hemorrhage in patients with syringomyelia
Summary
Gowers’ intrasyringeal hemorrhage is rare, this entity should be taken into consideration in patients with syringomyelia showing acute neurological deterioration. Surgical Neurology International 2014, 5:6 in childhood and had been diagnosed with Noonan syndrome on the basis of molecular genetic analysis She had short stature, webbing of the neck, low‐set ears, ocular hypertelorism, ptosis, and developmental delay. She had CM1 with cerebral hydrocephalus and a large syringomyelia extending from C1 level to the conus [Figure 1] She underwent foramen magnum decompression and a cervical syrinx‐subarachnoid shunt in 2003, a large syringeal cavity remained throughout the spinal cord. Magnetic resonance (MR) imaging showed a large syringomyelic cavity extending from the C1 level to the conus. Repeated MR imaging performed up to 3 years after the operation showed marked reduction of spinal cord diameter and shrinkage of the syringeal cavity [Figure 3]. There was peripheral hemosiderin deposition but no intramedullary neoplasms in the syringomyelic cavity
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