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Gowers' intrasyringeal hemorrhage associated with Chiari type I malformation in Noonan syndrome.

Mitsuhara T, Yamaguchi S, Takeda M, Kurisu K - Surg Neurol Int (2014)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Graduate School of Biomedical Sciences, Hiroshima University, 1-2-3 Kasumi, Minami.ku, Hiroshima 734-8551, Japan.

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.

No MeSH data available.


Related in: MedlinePlus

Initial magnetic resonance imaging in 2003 showing a large syringomyelic cavity extending from the C1 level to the conus (a and b). Head computed tomography and MR angiography reveal hydrocephalus and multiple aneurysms (c and d)
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Figure 1: Initial magnetic resonance imaging in 2003 showing a large syringomyelic cavity extending from the C1 level to the conus (a and b). Head computed tomography and MR angiography reveal hydrocephalus and multiple aneurysms (c and d)

Mentions: A 39-year-old female presented with progressive gait disturbance and urinary dysfunction of acute onset in 2010. She had been treated for tetralogy of Fallot 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 also had multiple untreated cerebral aneurysms. She had CM1 with cerebral hydrocephalus and a large syringomyelia extending from C1 level to the conus [Figure 1]. Although she underwent foramen magnum decompression and a cervical syrinx-subarachnoid shunt in 2003, a large syringeal cavity remained throughout the spinal cord.


Gowers' intrasyringeal hemorrhage associated with Chiari type I malformation in Noonan syndrome.

Mitsuhara T, Yamaguchi S, Takeda M, Kurisu K - Surg Neurol Int (2014)

Initial magnetic resonance imaging in 2003 showing a large syringomyelic cavity extending from the C1 level to the conus (a and b). Head computed tomography and MR angiography reveal hydrocephalus and multiple aneurysms (c and d)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3927095&req=5

Figure 1: Initial magnetic resonance imaging in 2003 showing a large syringomyelic cavity extending from the C1 level to the conus (a and b). Head computed tomography and MR angiography reveal hydrocephalus and multiple aneurysms (c and d)
Mentions: A 39-year-old female presented with progressive gait disturbance and urinary dysfunction of acute onset in 2010. She had been treated for tetralogy of Fallot 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 also had multiple untreated cerebral aneurysms. She had CM1 with cerebral hydrocephalus and a large syringomyelia extending from C1 level to the conus [Figure 1]. Although she underwent foramen magnum decompression and a cervical syrinx-subarachnoid shunt in 2003, a large syringeal cavity remained throughout the spinal cord.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Graduate School of Biomedical Sciences, Hiroshima University, 1-2-3 Kasumi, Minami.ku, Hiroshima 734-8551, Japan.

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.

No MeSH data available.


Related in: MedlinePlus