Hereditary spinal arachnoid cysts, distichiasis, and lymphedema

Schwartz, Jessica; O'Brien, Mark S.; Hoffman, James C.

doi:10.1002/ana.410070410

Cited by 61 publications

(32 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Interestingly, there were no cases of spinal extradural cysts (despite a number of published reports); however, these could be asymptomatic, as noted by Schwartz et al 26 Recently, a FOXC2 mutation has been described in a family with dominant inheritance of cleft palate and distichiasis. 27 Cleft palate occurred in three of our patients from different families.…”

Section: Discussionmentioning

confidence: 97%

Analysis of the phenotypic abnormalities in lymphoedema-distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24

Brice

2002

Journal of Medical Genetics

245

272

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 97%

Analysis of the phenotypic abnormalities in lymphoedema-distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24

Brice

2002

Journal of Medical Genetics

245

272

View full text Add to dashboard Cite

“…The etiology of these lesions is unclear; however, congenital, traumatic and inflammatory etiologies have been postulated. Published associations with congenital intradural arachnoid cysts include familial occurrence [1], hereditary distichiasis [2], neurocutaneous melanosis [3]and neural tube defects [4]. Herein, we report three cases with a congenital intradural arachnoid cyst of the spine.…”

Section: Introductionmentioning

confidence: 94%

Symptomatic Spinal Intradural Arachnoid Cysts in the Pediatric Age Group: Description of Three New Cases and Review of the Literature

Lee

Cho²

2001

Pediatr Neurosurg

View full text Add to dashboard Cite

Spinal arachnoid cysts are a relatively uncommon lesion that may be either intra- or extradural, and intradural spinal arachnoid cysts are even less common. These cysts are usually asymptomatic but may produce symptoms by compressing the spinal cord or nerve roots suddenly or progressively. We present three cases in the pediatric age group with spinal intradural arachnoid cysts without a preceding history of trauma. Three patients with symptomatic intradural arachnoid cysts were investigated with conventional T1- and T2-weighted magnetic resonance imaging (MRI). The MRI scans demonstrated the intradural arachnoid cysts with slightly lower CSF signal intensity on the gradient echo images and slightly higher signal intensity on T1-weighted images. The first cyst was located at the level T12–L1 and compressed the conus medullaris, with neurogenic bladder and cauda equina syndrome for 2 months. The second was located at the level C5–T1 ventrally, with spastic gait and neurogenic bladder for 4 years. The other was located at T2–3 ventrally, with sudden onset of quadriplegia after jumping rope. The combined treatment of total resection and wide fenestration in our three patients produced an excellent return of neurologic function in each one, except for residual urinary disturbance in case 2. Intradural spinal arachnoid cysts appear to result from an alteration of the arachnoid trabeculae; some such cysts are ascribed anecdotally to previous trauma or arachnoiditis, whereas the majority are idiopathic and congenital. The majority of intradural spinal arachnoid cysts occur in the thoracic region and most are dorsal to the neural elements. Only 10 cases have been reported in which the intradural arachnoid cysts were located anterior to the cervical spinal cord, of which 8 were in the pediatric age group, like our case 2. Myelography, postcontrast CT myelography and MRI have been demonstrated as useful for the diagnosis of intradural arachnoid cysts. MRI is the imaging modality of choice, and the extent, size and nature of the lesion in our cases were well demonstrated by MRI. Surgical treatment is necessary if progressive neurological dysfunction appears in the course of spinal cord compression. Complete surgical excision of the cysts is the best choice of treatment, and wide fenestration and shunting of the cyst to the peritoneum, pleural cavity or right atrium were the modalities of choice. MRI offers a noninvasive and effective means to make the diagnosis of arachnoid cysts easier. Intradural arachnoid cysts may cause progressive myelopathy; however, the postoperative prognosis is good if the operation is performed prior to neurologic deficits.

show abstract

“…If the cyst does not communicate with the subarachnoid space, complete excision can be performed without subsequent repair of the dural defect. Although some authors have advocated cystto-peritoneal shunting when the dural defect is large and not amenable to watertight repair, 18,19,23 we recommend a trial of patch grafting and fibrin glue repair before considering permanent shunt insertion. Figs.…”

Section: Neurosurg Focus / Volume 22 / February 2007mentioning

confidence: 99%

Spinal extradural arachnoid cysts: clinical, radiological, and surgical features

Liu

Cole

Kan

et al. 2007

FOC

106

154

View full text Add to dashboard Cite

✓Extradural arachnoid cysts in the spine are rare and are seldom a cause of spinal cord compression. They are thought to arise from congenital defects in the dura mater, and they almost always communicate with the intrathecal subarachnoid space through a small defect in the dura. The mainstay of treatment in patients with neurological symptoms is surgical removal of the cyst together with ligation of the communicating pedicle and closure of the dural defect. In the present paper the authors review the literature and discuss the clinical and pathological features, mechanisms of pathogenesis, neuroimaging characteristics, and surgical management of spinal extradural arachnoid cysts. The surgical technique for removal of these lesions is illustrated in a patient with a large thoracolumbar spinal extradural arachnoid cyst causing neurogenic claudication.

show abstract

Hereditary spinal arachnoid cysts, distichiasis, and lymphedema

Cited by 61 publications

References 6 publications

Analysis of the phenotypic abnormalities in lymphoedema-distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24

Analysis of the phenotypic abnormalities in lymphoedema-distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24

Symptomatic Spinal Intradural Arachnoid Cysts in the Pediatric Age Group: Description of Three New Cases and Review of the Literature

Spinal extradural arachnoid cysts: clinical, radiological, and surgical features

Contact Info

Product

Resources

About