Hydrocephalus due to idiopathic stenosis of the foramina of Magendie and Luschka

2003 ◽  
Vol 98 (4) ◽  
pp. 897-902 ◽  
Author(s):  
Carine Karachi ◽  
Caroline Le Guérinel ◽  
Pierre Brugières ◽  
Eliane Melon ◽  
Philippe Decq
Keyword(s):  
Fourth Ventricle ◽  
Third Ventricle ◽  
Ventricular Volume ◽  
Transverse Diameter ◽  
Type I ◽  
Content Type ◽  
Chiari Type I Malformation ◽  
Chiari Type I ◽  
Fine Print ◽  
Foramen Of Luschka

✓ Idiopathic stenosis of the foramina of Magendie and Luschka is a rare cause of obstructive hydrocephalus involving the four ventricles. Like other causes of noncommunicating hydrocephalus, it can be treated with endoscopic third ventriculostomy (ETV). Three patients who were 21, 53, and 68 years of age presented with either headaches (isolated or associated with raised intracranial pressure) or vertigo, or a combination of gait disorders, sphincter disorders, and disorders of higher functions. In each case, magnetic resonance (MR) imaging demonstrated hydrocephalus involving the four ventricles (mean transverse diameter of third ventricle 14.15 mm; mean sagittal diameter of fourth ventricle 23.13 mm; and mean ventricular volume 123.92 ml) with no signs of a Chiari Type I malformation (normal posterior fossa dimensions, no herniation of cerebellar tonsils). The diagnosis of obstruction was confirmed using ventriculography (in two patients) and/or MR flow images (in two patients). All patients presented with marked dilation of the foramen of Luschka that herniated into the cisterna pontis. All patients were treated using ETV. No complications were observed. All three patients became asymptomatic during the weeks following the surgical procedure and remained stable at a mean follow-up interval of 36 months. Postoperative MR images demonstrated regression of the hydrocephalus (mean transverse diameter of third ventricle 7.01 mm; mean sagittal diameter of fourth ventricle 16.6 mm; and mean ventricular volume 79.95 ml), resolution of dilation of the foramen of Luschka, and good patency of the ventriculostomy (flow sequences). These results confirm the existence of hydrocephalus caused by idiopathic fourth ventricle outflow obstruction without an associated Chiari Type I malformation, and the efficacy of ETV for this rare indication.

Spontaneous drainage of syringomyelia

1993 ◽  
Vol 79 (1) ◽  
pp. 132-134 ◽  
Author(s):  
Antonio Santoro ◽  
Roberto Delfini ◽  
Gualtiero Innocenzi ◽  
Claudio Di Biasi ◽  
Guido Trasimeni ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Mr Imaging ◽  
Clinical Course ◽  
Spontaneous Resolution ◽  
Type I ◽  
Content Type ◽  
Chiari Type I Malformation ◽  
Spinal Subarachnoid Space ◽  
Chiari Type I ◽  
Fine Print

✓ Two cases are reported of Arnold-Chiari type I malformation associated with syringomyelia, in which magnetic resonance (MR) imaging revealed spontaneous decompression of the syrinx. In one case axial MR imaging sections showed a communication between the syrinx and the spinal subarachnoid space, which supports the hypothesis that fissuring of the cord parenchyma is instrumental in the spontaneous resolution of syringomyelia. The MR imaging changes were not accompanied by variations in the patients' clinical course.

Chiari Type I malformation and syringomyelia in unrelated patients with blepharophimosis

1998 ◽  
Vol 89 (5) ◽  
pp. 835-838 ◽  
Author(s):  
Philippe Paquis ◽  
Michel Lonjon ◽  
Madeleine Brunet ◽  
Jean Claude Lambert ◽  
Patrick Grellier
Keyword(s):  
Autosomal Dominant ◽  
Radicular Pain ◽  
Hereditary Diseases ◽  
Type I ◽  
Content Type ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Familial Type ◽  
Chiari Type I ◽  
Fine Print

✓ Syringomyelia is a rare, mainly sporadic disease of the spinal cord, which is associated with 80% of cases in which a Chiari Type I malformation is also present. A mendelian transmission of syringomyelia (autosomal dominant or recessive) has been proposed in approximately 2% of reported cases. The association of syringomyelia with hereditary diseases (Noonan's syndrome, phacomatoses) has been mentioned frequently in the literature. The authors report the presence of a Chiari Type I malformation accompanied by syringomyelia in two unrelated patients affected by a familial Type II blepharophimosis—ptosis—epicanthus inversus syndrome (BPES). The first patient was a 35-year-old woman who presented with a right C-8 root paresia. The second case involved a 20-year-old man who complained of cervical radicular pain. Both belong to families in which BPES was segregated in an autosomal dominant modality, but other family members had no known neurological symptoms. To the authors' knowledge, such a combination has never been described. Perhaps the possible involvement of a genetic component in some cases of Chiari Type I—associated syringomyelia will someday be debated.

Hindbrain herniation: an unusual occurrence after shunting an intracranial arachnoid cyst

1994 ◽  
Vol 81 (1) ◽  
pp. 126-129 ◽  
Author(s):  
Maher I. Hassounah ◽  
Bengt E. Rahm
Keyword(s):  
Arachnoid Cyst ◽  
Type I ◽  
Content Type ◽  
Stereotactic Aspiration ◽  
Chiari Type I Malformation ◽  
Hindbrain Herniation ◽  
Intracranial Arachnoid Cyst ◽  
Unusual Occurrence ◽  
Chiari Type I ◽  
Fine Print

✓ The case is presented of a prepontine arachnoid cyst in a 28-year-old woman. She was treated by craniotomy, stereotactic aspiration, internal shunting, and finally by insertion of a cystoperitoneal shunt. Eight months later she developed an asymptomatic Arnold-Chiari type I malformation. The pathogenesis of this unusual hindbrain herniation is explained on the basis of the differential pressure between two compartments and cephalocranial disproportion.

Syringomyelia extending to the basal ganglia

1989 ◽  
Vol 71 (4) ◽  
pp. 616-617 ◽  
Author(s):  
Satoshi Okada ◽  
Yoshio Nakagawa ◽  
Kimiyoshi Hirakawa
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pyramidal Tract ◽  
Type I ◽  
Sensory Disturbance ◽  
Resonance Imaging ◽  
Content Type ◽  
Chiari Type I Malformation ◽  
The Right ◽  
Chiari Type I ◽  
Fine Print

✓ A 10-year-old girl was admitted to the hospital with complaints of progressive right hemiparesis and sensory disturbance. Magnetic resonance imaging revealed a Chiari Type I malformation and syringomyelia from T-10 to C-1. The syrinx extended from the medulla to the right putamen along the pyramidal tract.

A novel fibroblast growth factor receptor 2 mutation in Crouzon syndrome associated with Chiari Type I malformation and syringomyelia

2002 ◽  
Vol 97 (2) ◽  
pp. 396-400 ◽  
Author(s):  
Hironori Fujisawa ◽  
Mitsuhiro Hasegawa ◽  
Shinya Kida ◽  
Junkoh Yamashita
Keyword(s):  
Fibroblast Growth Factor Receptor ◽  
Growth Factor Receptor ◽  
Type I ◽  
Crouzon Syndrome ◽  
Content Type ◽  
Chiari Type I Malformation ◽  
Lambdoid Synostosis ◽  
Chiari I ◽  
Chiari Type I ◽  
Fine Print

Object. It has been reported that due to premature synostosis of the lambdoid suture in the first 24 months of life, more than 70% of patients with Crouzon syndrome concurrently suffer from chronic tonsillar herniation (Chiari Type I malformation) and some (20%) associated syringomyelia. The goal of the present study was to examine mutations in the fibroblast growth factor receptor (FGFR) genes in Crouzon syndrome and its related conditions. Methods. Five patients were studied: three with Crouzon syndrome (one sporadic and two familial), one with sporadic Chiari I with syringomyelia, and one with unilateral lambdoid synostosis. Deoxyribonucleic acid was screened for FGFR1–3 mutations by using single-strand conformational polymorphism and subsequent direct sequencing. Two types of missense mutations were detected in the FGFR2 gene, Cys342Trp (1205, TGC → TGG) in a patient with sporadic Crouzon syndrome and Tyr281Cys (1021, TAC → TGC) in two siblings (brother and sister) with familial Crouzon syndrome, respectively. The former has been reported only in sporadic cases but the latter has not previously been identified. A polymorphism in the FGFR3 gene, Asn294Asn (882, AAT → AAC), was also detected in three patients. No mutation was found in the patient with sporadic Chiari I with syringomyelia. Conclusions. The FGFR2 missense mutation was detected in Crouzon syndrome but not in sporadic Chiari I with syringomyelia or lambdoid synostosis. A novel FGFR2 mutation, Tyr281Cys, was found in familial Crouzon syndrome with Chiari I and syringomyelia. It may be informative to look for this in patients with Crouzon syndrome and associated syringomyelia.

Chiari I malformation: a rare cause of noncommunicating hydrocephalus treated by third ventriculostomy

2001 ◽  
Vol 95 (5) ◽  
pp. 783-790 ◽  
Author(s):  
Philippe Decq ◽  
Caroline Le Guérinel ◽  
Jean-Christophe Sol ◽  
Pierre Brugières ◽  
Michel Djindjian ◽  
...  
Keyword(s):  
Third Ventricle ◽  
Chiari I Malformation ◽  
Third Ventriculostomy ◽  
Transverse Diameter ◽  
Content Type ◽  
The Third ◽  
Chiari I ◽  
Noncommunicating Hydrocephalus ◽  
Fine Print

Object. Hydrocephalus associated with Chiari I malformation is a rare entity related to an obstruction in the flow of cerebrospinal fluid (CSF) in the foramen of Magendie. Like all forms of noncommunicating hydrocephalus, it can be treated by endoscopic third ventriculostomy (ETV). The object of this study is to report a series of five cases of hydrocephalus associated with Chiari I malformation and to evaluate the use of ETV in the treatment of this anomaly. Methods. Five patients (four women and one man with a mean age of 29.6 years) underwent ETV for hydrocephalus associated with Chiari I malformation between April 1991 and February 1997. All patients had presented with paroxysmal headaches, which in two cases were associated with visual disorders. All patients had also presented with hydrocephalus (mean transverse diameter of the third ventricle 12.79 mm; mean sagittal diameter of the fourth ventricle 18.27 mm) with a mean herniation of the cerebellar tonsils at 13.75 mm below the basion—opisthion line. Surgery was performed in all patients by using a rigid endoscope. No complications occurred either during or after the procedure, except in one patient who experienced a wound infection that was treated by antibiotic medications. The mean duration of follow up in this study was 50.39 months. Four patients became completely asymptomatic and remained stable throughout the follow-up period. One patient required an additional third ventriculostomy after 1 year, due to secondary closure, and has remained stable since that time. Postoperative magnetic resonance images demonstrated a significant reduction in the extent of hydrocephalus in all patients (mean transverse diameter of the third ventricle 6.9 mm [p = 0.0035]; mean sagittal diameter of the fourth ventricle 10.32 mm [p = 0.007]), with a mean ascent of the cerebellar tonsils from 13.75 mm below the basion—opisthion line to 7.76 mm below it (p = 0.01). In addition, CSF flow was identified on either side of the orifice of the third ventriculostomy in all patients postoperatively. Conclusions. Results in this series confirm the efficacy of ETV in the treatment of hydrocephalus associated with Chiari I malformation. It is a reliable, minimally invasive technique that also provides a better understanding of the pathophysiology of this malformation.

Comparison of the transvermian and telovelar approaches to the fourth ventricle

2004 ◽  
Vol 101 (3) ◽  
pp. 484-498 ◽  
Author(s):  
Necmettin Tanriover ◽  
Arthur J. Ulm ◽  
Albert L. Rhoton ◽  
Alexandre Yasuda
Keyword(s):  
Fourth Ventricle ◽  
Image Guidance ◽  
Surgical Approaches ◽  
Content Type ◽  
Lateral Recess ◽  
Telovelar Approach ◽  
Additional Exposure ◽  
Formalin Fixed ◽  
Fine Print ◽  
Foramen Of Luschka

Object. The two most common surgical routes to the fourth ventricle are the transvermian and telovelar approaches. The purpose of this study was to compare the microanatomy and exposures gained through these approaches. Methods. Ten formalin-fixed specimens were dissected in a stepwise manner to simulate the transvermian and telovelar surgical approaches. Stealth image guidance was used to compare the exposures and working angles obtained using these approaches. The transvermian and telovelar approaches provided access to the entire rostrocaudal length of the fourth ventricle floor from the aqueduct to the obex. In addition, both approaches provided access to the entire width of the floor of the fourth ventricle. The major difference between the two approaches regarded the exposure of the lateral recess and the foramen of Luschka. The telovelar, but not the transvermian, approach exposed the lateral and superolateral recesses and the foramen of Luschka. The transvermian approach, which offered an incision through at least the lower third of the vermis, afforded a modest increase in the operator's working angle compared with the telovelar approach when accessing the rostral half of the fourth ventricle. Conclusions. The transvermian approach provides slightly better visualization of the medial part of the superior half of the roof of the fourth ventricle. The telovelar approach, which lacks incision of any part of the cerebellum, provides an additional exposure to the lateral recesses and the foramen of Luschka.

Microsurgical removal of paraventricular cavernous angiomas

1981 ◽  
Vol 55 (2) ◽  
pp. 308-311 ◽  
Author(s):  
Eugenio Pozzati ◽  
Giulio Gaist ◽  
Massimo Poppi ◽  
Bernardino Morrone ◽  
Roberto Padovani
Keyword(s):  
Fourth Ventricle ◽  
Third Ventricle ◽  
Cavernous Angioma ◽  
The Other ◽  
Content Type ◽  
Cavernous Angiomas ◽  
The Third ◽  
Microsurgical Approach ◽  
The Right ◽  
Fine Print

✓ Two cases of paraventricular cavernous angiomas are presented. In one, the cavernous angioma was found in the right wall of the fourth ventricle, and in the other in the right thalamus encroaching upon the third ventricle. Both patients had onset of symptoms suggesting a tumor. Good results were obtained by the microsurgical approach to these malformations. The computerized tomography findings typical of cavernous angiomas are reviewed.

Conray ventriculography in the diagnosis of infantile hydrocephalus

1973 ◽  
Vol 39 (6) ◽  
pp. 780-783 ◽  
Author(s):  
Hiroshi Yamada ◽  
Masataka Tajima
Keyword(s):  
Contrast Medium ◽  
Fourth Ventricle ◽  
Third Ventricle ◽  
Infantile Hydrocephalus ◽  
Content Type ◽  
The Third ◽  
Aqueduct Of Sylvius ◽  
Conray Ventriculography ◽  
Fine Print

✓ A method for visualizing the third ventricle, aqueduct of Sylvius, and fourth ventricle with methylglucamine iothalamate 60% (Conray) in infantile hydrocephalus is described. Only a small amount of contrast medium is necessary to visualize these structures. This procedure has been performed in 47 infants from 7 days to 3 years of age, and has been proved simple, safe, and valuable in the diagnosis of lesions around the third and fourth ventricles.

Calculation of the Volume of Fourth Ventricle and the Length of Herniation Using the ImageJ Software in Patients with Chiari Type I malformation

2012 ◽  
Vol 19 (4) ◽  
pp. 249-255
Author(s):  
Tolga Ertekin ◽  
Niyazi Acer ◽  
Umit Erkan Vurdem ◽  
Erdogan Unur ◽  
Mahmut Cay
Keyword(s):  
Fourth Ventricle ◽  
Type I ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Imagej Software ◽  
Chiari Type I
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